Intestinal pseudo-obstruction in systemic lupus erythematosus: an uncommon but important clinical manifestation.
Mok M Y,Wong R W,Lau C S
Lupus
OBJECTIVES:To document intestinal pseudo-obstruction (IpsO) as a recognised clinical manifestation of systemic lupus erythematosus (SLE) and a possible new clinical entity with its apparent association with ureterohydronephrosis. METHODOLOGY:We report six lupus patients who presented with IpsO and review 12 other cases from an English literature search. IpsO is defined as the presence of clinical features suggestive of intestinal obstruction but without organic obstruction, namely absence of bowel sounds, presence of multiple fluid levels on plain abdominal X-rays and exclusion of organic obstruction by imaging or surgical procedure. Other clinical characteristics related to the underlying lupus, serological and histological findings, treatment modalities and outcomes of these patients were reviewed. RESULTS:All 18 patients fulfilled the ACR revised classification criteria for SLE. None showed any clinical features of scleroderma or overlap syndrome. The mean age of onset of IpsO was 29.0 (15-47) y. The female to male ratio was 16:2. Nine patients had IpsO as the initial presentation of their underlying lupus. Coexisting lupus involvement of other organ systems included glomerulonephritis (n=7), thrombocytopenia (n=5) and cerebral lupus (n=3). The serology data and autoantibody profile of some of the previously reported patients were incomplete. In our series, anti-Ro antibody was positive in 5/6 while anti-RNP was found in 1/6 patients only. All our patients had active lupus serology at presentation. 17/18 patients required the use of high dose systemic corticosteroid therapy while one patient responded to topical adrenocorticotrophin hormone treatment. Response was good and was observed early after commencement. Azathioprine was used as maintenance therapy in 6/18 patients with good effects. An apparent association with the presence of bilateral ureterohydronephrosis was found in 12/18 patients. These patients presented with dysuria without positive bacterial culture though features of chronic interstitial cystitis were not invariably found in these patients. CONCLUSION:IpsO is an uncommon but important manifestation of SLE. The underlying pathology is not fully understood but it may be related to immune complex deposition. The finding of coexisting ureterohydronephrosis suggests that there may also be a central smooth muscle motility problem of neuropathic or myogenic pathophysiology which may or may not be secondary to vasculitis. Early recognition and treatment of IpsO in SLE is important.
10.1177/096120330000900104
Acute lupus pneumonitis followed by intestinal pseudo-obstruction in systemic lupus erythematosus: A case report.
Ji Caihong,Yu Xing,Wang Yong,Shi Lufeng
Experimental and therapeutic medicine
Intestinal pseudo-obstruction (IpsO) and acute lupus pneumonitis (ALP) are uncommon severe complications of systemic lupus erythematosus (SLE). The present study reports the case of a 26-year-old female who presented with abdominal pain, nausea and vomiting as initial symptoms. Computed tomography (CT) scanning revealed the jejunal wall was thickened and streaky, mimicking the presentation of intestinal obstruction. Following emergency surgery, the patient's general condition was aggravated, with evident limb erythematous rashes. A series of laboratory examinations revealed SLE, and combined with patient's medical history IpsO was diagnosed, with a disease Activity Index score of 10. During the therapeutic period, high fever, dyspnea and oxygen saturation (SaO) reductions were detected, and CT scans indicated lung infiltration, excluding other causes through a comprehensive infectious work-up and a bronchoalveolar lavage examination. ALP was confirmed and treated with high-dose methylprednisolone and gamma globulin supplement. The patient responded well and was discharged in 2 weeks. In the one-year tapering period and after stopping corticosteroids, the patient recovered well with no relapse detected. In conclusion, the manifestation of IpsO in SLE is rare and represents a challenge for the surgeon to establish the correct diagnosis and avoid inappropriate surgical intervention. ALP may be the consequence of emergency surgery, and immediate high-dose glucocorticoid therapy is recommended.
10.3892/etm.2016.3316
A frequently missed entity in systemic lupus erythematosus (SLE): Intestinal pseudo-obstruction (IpsO).
Shirley L,Thundyil R J
The Medical journal of Malaysia
Intestinal pseudo-obstruction (IpsO) is defined as presence of clinical features of intestinal obstruction without identifiable mechanical obstructive lesion. IpsO is an uncommon gastrointestinal manifestation of systemic lupus erythematosus (SLE) and is largely under-recognised. There are only over 30 published cases in English literature on SLE-related IpsO. Herein, we report two cases of SLE-related IpsO to illustrate the importance of early recognition to avoid unnecessary surgical intervention, as SLE-related IpsO responds well to systemic high dose corticosteroids. These two cases also demonstrate the apparent association of IpsO with uretero-hydronephrosis, suggesting that the possible mechanism could be smooth muscle dysmotility.
Intestinal pseudo-obstruction in patients with systemic lupus erythematosus: a real diagnostic challenge.
García López Carlos Alberto,Laredo-Sánchez Fernando,Malagón-Rangel José,Flores-Padilla Miguel G,Nellen-Hummel Haiko
World journal of gastroenterology
Intestinal pseudo-obstruction secondary to systemic lupus erythematosus (SLE) is a rare syndrome described in recent decades. There are slightly over 30 published cases in the English language literature, primarily associated with renal and hematological disease activity. Its presentation and evolution are a diagnostic challenge for the clinician. We present four cases of intestinal pseudo-obstruction due to lupus in young Mexican females. One patient had a previous diagnosis of SLE and all presented with a urinary tract infection of varying degrees of severity during their evolution. We consider that recognition of the disease is of vital importance because it allows for establishing appropriate management, leading to a better prognosis and avoiding unnecessary surgery and complications.
10.3748/wjg.v20.i32.11443
Predictors of intestinal pseudo-obstruction in systemic lupus erythematosus complicated by digestive manifestations: data from a Southern China lupus cohort.
Huang Q,Lai W,Yuan C,Shen S,Cui D,Zhao J,Lin J,Ren H,Yang M
Lupus
OBJECTIVE:To determine factors that may predict intestinal pseudo-obstruction (IpsO) in systemic lupus erythematosus (SLE) patients complicated by digestive manifestations. METHODS:SLE patients with digestive manifestations (n = 135) were followed at Southern Medical University affiliated Nanfang Hospital from 2000 until 2013. Demographic variables, clinical features, and laboratory data were compared between the two groups. Univariate and multivariate logistic regression models were used to establish factors that predispose to IpsO in these patients. RESULTS:At the end of the study period, 32 (23.7%) patients had developed IpsO. Mortality (9 patients) was infrequent and the cause of death was unrelated to IpsO. Independent predictors of IpsO in SLE were ureterectasia, anti-U1 RNP(+), peritonitis, and low C3 levels. CONCLUSIONS:Regular abdominal X-ray examinations are recommended in SLE patients with ureterectasia, anti-U1 RNP(+), peritonitis, or low C3 levels, as early diagnosis and therapy may prevent unnecessary surgical intervention and improve the disease course.
10.1177/0961203315605366
Intestinal pseudo-obstruction in systemic lupus erythematosus.
Narváez J,Pérez-Vega C,Castro-Bohorquez F J,Garcia-Quintana A M,Biosca M,Vilaseca-Momplet J
Scandinavian journal of rheumatology
In order to document intestinal pseudo-obstruction (IPO) as a recently recognized manifestation of systemic lupus erythematosus (SLE), we report the case or a woman with SLE who presented with IPO and we review 21 other previously reported cases from an English literature search. In 41% of the cases, IPO was the initial manifestation of their underlying lupus. The clinical and laboratory features were not significantly different from those reported in large series of patients with SLE, except for an apparent association with an urinary tract involvement (ureterohydronephrosis and interstitial cystitis). The pathogenic mechanism of this complication is not fully understood, but seems to be heterogeneous. IPO responded readily to high dose steroid therapy in all patients, but in some cases this complication evolved regardless of the underlying disease activity. A high level of awareness of this complication is needed to avoid unnecessary surgical intervention.
Intestinal pseudo-obstruction syndrome in systemic lupus erythematosus.
Zhang J,Fang M,Wang Y,Mao J,Sun X
Lupus
Intestinal pseudo-obstruction (IpsO) occurs in a small subgroup of patients with systemic lupus erythematosus (SLE), and has been under-recognized. The etiology and mechanisms are unclear. Herein, we report two cases. In case 1, IpsO occurred as the complication in a previously diagnosed SLE patient. In case 2, IpsO with an image-evidenced intussusception and a peripheral eosinophilia, was the initial presentation, leading to the diagnosis of SLE. In both cases, IpsO existed concomitantly with ureterohydronephrosis, and evolved into peritonitis, which we called together IpsO syndrome. However, this surgical abdomen-like syndrome had a satisfactory response to glucocorticosteroids instead of antibiotics and surgery. Physician awareness and early diagnosis could positively affect outcomes of this syndrome, avoiding unnecessary surgical intervention.
10.1177/0961203311405702
Intestinal pseudo-obstruction in systemic lupus erythematosus: a case report and review of the literature.
Wang Jian-Lin,Liu Gang,Liu Tong,Wei Jiang-Peng
Medicine
Intestinal pseudo-obstruction (IPO) is a rare but dangerous complication of systemic lupus erythematosus (SLE) when the patient has no other manifestations except gastrointestinal symptoms. We performed 1 patient with a 2-month history of recurrent vomiting and abdominal distension. She admitted past surgical histories of cesarean section and appendectomy. A physical examination revealed tenderness in the right lower abdominal on palpation and bowel sounds were weak, 2 to 3 bpm. An x-ray and CT of her abdomen showed intestinal obstruction. The initial diagnosis was adhesive intestinal obstruction. She received surgical treatment because her symptoms had gradually become more frequent and persistent. But she vomited again 2 weeks later after the surgery. Further immunology tests indicated that she had an IPO secondary to SLE. We treated the patient with methylprednisolone pulse for 3 days and followed by prednisone orally. The patient had a good response. Complete remission was achieved on 8 years follow-up. The importance of IPO secondary to SLE lies in an early diagnosis. After the diagnosis is established, immunosuppressive therapy should be the initial and first-line treatment, and surgical intervention is often disappointing and should be carefully avoided. It is necessary to enhance awareness of doctors to IPO secondary to SLE.
10.1097/MD.0000000000000248
A Report of Chronic Intestinal Pseudo-obstruction Related to Systemic Lupus Erythematosus.
Wang Ruiqiang,Zheng Bowen,Wang Biyue,Ma Pupu,Chen Fengmei,Tang Lin
Open medicine (Warsaw, Poland)
Chronic intestinal pseudo-obstruction (CIPO) is a functional gastrointestinal disorder with symptoms of ileus. CIPO can either be idiopathic or secondary to other diseases such as systemic lupus erythematosus (SLE). SLE is involved in many parts of the gastrointestinal system with variable clinical presentations. Reports about reduplicated CIPO as a complication of SLE is infrequent. A 49-year-old female suffering from clinical symptoms of ileus has been hospitalized 3 times over 1 year. Her examination results showed no observation of mechanical obstruction. In August 2017, she came to the nephrology department due to edema in both lower limbs along with symptoms of ileus. After thorough examination, she was diagnosed with secondary CIPO related to SLE. Results of renal biopsy confirmed to be lupus nephritis (Class III-(A) + V). The symptoms of ileus are gradually improved after treatment of full-dose intravenous corticosteroid for 5 days.
10.1515/med-2018-0083
Chronic intestinal pseudo-obstruction: a rare first manifestation of systemic lupus erythematosus.
Khairullah S,Jasmin R,Yahya F,Cheah T E,Ng C T,Sockalingam S
Lupus
Chronic intestinal pseudo-obstruction (CIPO) is a rare clinical syndrome of ineffective intestinal motility characterised by clinical and radiological evidence of intestinal obstruction with no identifiable mechanical lesion. CIPO can either be idiopathic or secondary to a systemic disease, like systemic lupus erythematosus (SLE). Fewer than 30 cases of CIPO secondary to SLE have been reported so far. Here we describe a case of SLE with the initial presentation of CIPO. In SLE-related CIPO, treatment includes a combination of high-dose intravenous corticosteroids, immunosuppressants and supportive care. With awareness of this condition, unnecessary surgical intervention and repeated invasive procedures could be avoided. Early initiation of treatment would avoid complications and bring about resolution of symptoms.
10.1177/0961203313492873
Intestinal Pseudo-Obstruction as an Initial Manifestation of Systemic Lupus Erythematosus.
Oh Dong Jun,Yang Jae Nam,Lim Yun Jeong,Kang Ji Hyuk,Park Jung Hyun,Kim Mal Young
Intestinal research
Intestinal pseudo-obstruction (IPO) is an uncommon, severe complication that occurs in a small subgroup of patients with systemic lupus erythematosus (SLE). To our knowledge, approximately 30 cases of IPO in SLE have been reported in the literature. Moreover, IPO is rare as an initial manifestation of SLE. We report a case of a 43-year-old woman with SLE who initially presented with IPO.
10.5217/ir.2015.13.3.282
Intestinal pseudo-obstruction in systemic lupus erythematosus complicated by Castleman disease: a case report.
Annals of translational medicine
Background:Systemic lupus erythematosus (SLE) is a systemic disease, which can bring damage to multiple organ systems. It is easily misdiagnosed as mechanical intestinal obstruction and treated by surgery, which not only brings physical pain to patients, but also increases their economic burden. On the other hand, Castleman disease (CD) is also a rare disease that can be easily missed clinically. Consequently, IPO in SLE complicated by CD is extremely rare in clinical practice and easily ignored for clinicians, which may result in delayed diagnosis, and treatment, and even overtreatment. Case Description:An 18-year-old Chinese woman presented with over a month's history of abdominal pain and fever, accompanied by abdominal distension and nausea. The patient was admitted to a local hospital before admission, and imaging test showed intestinal obstruction. After symptomatic treatment, abdominal pain was relieved, but symptoms reappeared about 20 days later. In addition, a red rash on face, light-sensitiveness and alopecia appeared 7 months prior to presentation. Physical examination showed a temperature of 38.9 ℃, facial butterfly erythema, enlarged axillary lymph nodes, lower abdominal tenderness, and diminished bowel sounds. Laboratory examinations showed proteinuria, decreased white blood cell, low C3 and low C4, positive antinuclear antibody (ANA), and positive anti-double-stranded DNA (anti-dsDNA). Abdominal noncontrast computed tomography (CT) showed partial small bowel obstruction. Chest contrast-enhanced CT showed multiple enlarged lymph nodes in the bilateral axillary and mediastinal areas. The results of the axillary lymph node biopsy were consistent with the typical histologic features of clear vascular CD. After glucocorticoid and immunosuppression therapy, the patient's immune indexes and proteinuria gradually returned to normal and abdominal pain did not recur during the follow up. Conclusions:In order to avoid misdiagnosis of IPO in SLE and missed diagnosis of SLE complicated by CD, this case emphasizes the importance of medical history combined with appropriate laboratory examination, imaging examination and lymph node biopsy in SLE patients with lymphadenopathy for accurate diagnosis and reasonable treatment. At the same time, this case report aims to improve the diagnostic thinking of clinicians for similar patients.
10.21037/atm-22-4461
Intestinal Pseudo-Obstruction - An Under-Recognized Presentation of Systemic Lupus Erythematosus.
Indian journal of nephrology
Intestinal pseudo-obstruction (IPO), characterized by obstruction without an identifiable anatomic cause, is a rare and poorly understood entity that may occur as a primary condition or secondary to other autoimmune disorders such as systemic lupus erythematosus (SLE). A12-year-old female child was brought with abdominal distension, vomiting, and fever for 15 days. Examination showed height and weight less than the third centile for age, tachypnea, tachycardia, and hypertension with severe abdominal distension, copious bilious aspirate, and very sluggish bowel sounds. Abdominal X-ray showed multiple air fluid levels. Ultrasound abdomen and unenhanced computed tomography (CT) scan revealed thickened dilated bowel loops, ascites, and pleural effusion. In view of multisystem nature of the disease, Koch's abdomen or autoimmune disease was suspected and emergency laparotomy procedure was deferred. She was evaluated and diagnosed to have SLE with lupus nephritis class V as per the International Society of Nephrology/Royal Pathology Society. She was managed conservatively with nasogastric decompression, immunosuppressive therapy and supportive hemodialysis.
10.4103/ijn.IJN_494_20
Intestinal pseudo-obstruction as the initial manifestation of systemic lupus erythematosus.
Zhang Fang-Jie,Zhang Juan,Zhou Li-Ping,Wang Ai-Min,Li Xiang-Min
The American journal of emergency medicine
Small bowel obstruction is common in emergency departments. However, the exact cause of intestinal pseudo-obstruction (IPO) is often misdiagnosed. IPO is considered a severe manifestation of systemic lupus erythematosus (SLE). However, IPO is rare as the initial manifestation of SLE. This paper reports a female patient who presented with IPO as the initial manifestation and was ultimately diagnosed with SLE. The 31-year-old female was definitively diagnosed with SLE after IPO symptoms for 1 month. She then presented multiple organ dysfunction syndrome (MODS) leading to a poor prognosis. Patients with unexplained SBO symptoms should be aware of systemic diseases. Early diagnosis and prompt medical treatment are crucial to avoid unnecessary surgery and obtain satisfactory outcomes.
10.1016/j.ajem.2018.09.048
Intestinal pseudo-obstruction in systemic lupus erythematosus: an analysis of nationwide inpatient sample.
Clinical rheumatology
Intestinal pseudo-obstruction (IPO) is a rare gastrointestinal complication of systemic lupus erythematosus (SLE). We aimed to determine the prevalence and in-hospital outcomes of SLE-related intestinal pseudo-obstruction (SLE-IPO) using a large national database. This is a retrospective study using Nationwide Inpatient Sample (NIS) from 2016 to 2018. We included adult patients with SLE using International Classification of Diseases 10th Revision (ICD-10) code M32 in combination with codes for IPO, and we determined the in-hospital mortality, occurrence of sepsis, length of hospitalization, use of total parenteral nutrition (TPN), and frequency of surgical gastrointestinal procedures. The prevalence of IPO among hospitalized adult SLE patients was 1.7%, and the in-hospital mortality of SLE-IPO was 5.04%. Compared with SLE patients without IPO (group 2), SLE-IPO patients had higher in-hospital mortality (OR 2.07, 95%CI 1.60-2.64), higher risk of sepsis (OR 1.63, 95%CI 1.42-1.87), longer in-hospital stay (β 3.09, 95%CI 2.76-3.42), and higher frequency of TPN (OR 16.20, 95%CI 11.62-22.21) and gastrointestinal procedures (OR 5.47, 95%CI 4.62-6.43). SLE-IPO was associated with increased risk of sepsis (OR 1.29, 95%CI 1.12-1.49), compared with idiopathic IPO patients (group 3), and a possible increase in the frequency of gastrointestinal procedures (OR 1.14, 95%CI 0.96-1.34) compared with IPO secondary to diabetes mellitus (group 4), although not statistically significant. SLE-IPO patients were at twofold risk of in-hospital mortality compared with SLE patients without IPO. SLE-IPO was also associated with longer in-hospital stay and increased frequency of TPN and gastrointestinal procedures. Key Points • The prevalence of IPO in adult SLE patients was 1.7% in this US-based database. • In-hospital mortality rate of SLE-IPO was 4.95%. SLE-IPO patients were associated with twofold risk of in-hospital mortality compared with those without IPO. • SLE-IPO patients were also at increased risk of sepsis, and they had higher rate of TPN and gastrointestinal procedures.
10.1007/s10067-022-06283-z
Gastrointestinal and Hepatic Disease in Systemic Lupus Erythematosus.
Brewer Brian N,Kamen Diane L
Rheumatic diseases clinics of North America
Gastrointestinal (GI) symptoms are common among patients with systemic lupus erythematosus (SLE), although only rarely are they caused by active organ system involvement from SLE itself. Rapid diagnosis and appropriate treatment of lupus enteritis and other GI manifestations of SLE are critical, because of the potential for organ and life-threatening complications. The 3 main variants of lupus enteritis are lupus mesenteric vasculitis, intestinal pseudo-obstruction, and protein-losing enteropathy. These GI manifestations and others in patients with SLE are reviewed here.
10.1016/j.rdc.2017.09.011
Gastrointestinal system involvement in systemic lupus erythematosus.
Li Z,Xu D,Wang Z,Wang Y,Zhang S,Li M,Zeng X
Lupus
Systemic lupus erythematosus (SLE) is a multisystem disorder which can affect the gastrointestinal (GI) system. Although GI symptoms can manifest in 50% of patients with SLE, these have barely been reviewed due to difficulty in identifying different causes. This study aims to clarify clinical characteristics, diagnosis and treatment of the four major SLE-related GI system complications: protein-losing enteropathy (PLE), intestinal pseudo-obstruction (IPO), hepatic involvement and pancreatitis. It is a systematic review using MEDLINE and EMBASE databases and the major search terms were SLE, PLE, IPO, hepatitis and pancreatitis. A total of 125 articles were chosen for our study. SLE-related PLE was characterized by edema and hypoalbuminemia, with Technetium 99m labeled human albumin scintigraphy (Tc HAS) and alpha-1-antitrypsin fecal clearance test commonly used as diagnostic test. The most common site of protein leakage was the small intestine and the least common site was the stomach. More than half of SLE-related IPO patients had ureterohydronephrosis, and sometimes they manifested as interstitial cystitis and hepatobiliary dilatation. Lupus hepatitis and SLE accompanied by autoimmune hepatitis (SLE-AIH overlap) shared similar clinical manifestations but had different autoantibodies and histopathological features, and positive anti-ribosome P antibody highly indicated the diagnosis of lupus hepatitis. Lupus pancreatitis was usually accompanied by high SLE activity with a relatively high mortality rate. Early diagnosis and timely intervention were crucial, and administration of corticosteroids and immunosuppressants was effective for most of the patients.
10.1177/0961203317707825
A review of 42 cases of intestinal pseudo-obstruction in patients with systemic lupus erythematosus based on case reports.
Jin Peng,Ji Xiaoyan,Zhi Haining,Song Xiaodong,Du Haiwei,Zhang Kai,Shao Rongjiang,Ge Shanyi,Chen Qinghai,Lu Hongliang,Lu Junhua
Human immunology
Intestinal pseudo-obstruction (IpsO) is considered a severe manifestation of systemic lupus erythematosus (SLE) characterized by clinical and radiological evidence of intestinal obstruction with no identifiable mechanical lesion. We performed a systematic review to document IpsO in SLE. Twenty-eight articles with 42 patients were included. The median age of onset of IpsO was 27.5 (10-57) years. The female to male ratio was 38:4. Twenty-two (52.4%) patients had IpsO as the initial presentation of their underlying lupus. Three (7.1%) patients manifested in inactive lupus. The duration of abdominal symptoms before admitted ranged from 3 days to 3 years, however most of the patients responded well to systemic corticosteroid or immunosuppressive treatment within 2 days to about 3 months. Concomitant ureterohydronephrosis was present in approximately three-fourths of the cases. More interestingly, 4 patients presented hepatobiliary dilatation without mechanical obstruction together with IPO and ureterohydronephrosis. In conclusion, IpsO is an uncommon but important manifestation of SLE. The finding of coexisting ureterohydronephrosis and hepatobiliary dilatation suggests that there may be generalized visceral muscle dysmotility. Early recognition of IpsO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.
10.1016/j.humimm.2015.09.022
Chronic intestinal pseudo-obstruction in systemic lupus erythematosus.
Perlemuter G,Chaussade S,Wechsler B,Cacoub P,Dapoigny M,Kahan A,Godeau P,Couturier D
Gut
BACKGROUND/AIMS:Chronic intestinal pseudo-obstruction (CIPO) reflects a dysfunction of the visceral smooth muscle or the enteric nervous system. Gastrointestinal manifestations are common in systemic lupus erythematosus (SLE) but CIPO has not been reported. Features of CIPO are reported in five patients with SLE. METHODS:From 1988 to 1993, five patients with SLE or SLE-like syndrome were hospitalised for gastrointestinal manometric studies. CIPO was the onset feature in two cases. Antroduodenal manometry (three hours fasting, two hours fed) was performed in all patients, and oesophageal manometry in four. RESULTS:Intestinal hypomotility associated with reduced bladder capacity and bilateral ureteral distension was found in four patients and aperistalsis of the oesophagus in three. Treatment, which consisted of high dose corticosteroids, parenteral nutrition, promotility agents, and antibiotics, led to remission of both CIPO and urinary abnormalities in all cases. Antroduodenal manometry performed in two patients after remission showed increased intestinal motility. One patient died, and postmortem examination showed intestinal vasculitis. CONCLUSIONS:CIPO in SLE is a life threatening situation that can be reversed by treatment. It may be: (a) a complication or onset feature of the disease; (b) secondary to smooth muscle involvement; (c) associated with ureteral and vesical involvement; (d) the result of intestinal vasculitis.
10.1136/gut.43.1.117
Gastrointestinal Involvement in Children with Systemic Lupus Erythematosus.
Children (Basel, Switzerland)
Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder. When it presents before the age of 18 years (childhood-onset systemic lupus erythematosus, cSLE), the disease course tends to be more severe with a higher rate of organ involvement and requires an early diagnosis. Gastrointestinal involvement in cSLE is rare and scarcely reported in the literature. Any organ of the gastrointestinal system may be affected, either as a direct consequence of the disease, as a subsequent complication, or as an adverse drug event. Abdominal pain is the most common GI symptom, it can be diffuse or well localized, and can underline different conditions such as hepatitis, pancreatitis, appendicitis, peritonitis, or enteritis. cSLE may have an alteration of the intestinal barrier with features of protein-losing enteropathy or, in genetically predisposed patients, may develop associated autoimmune disorders such as Coeliac Disease or Autoimmune Hepatitis. The aim of this manuscript is to provide a narrative review of gastrointestinal manifestations in cSLE focused on hepatic, pancreatic, and intestinal involvement. A comprehensive literature search based on the PubMed database was performed.
10.3390/children10020309
Clinical characteristics of lupus enteritis in Japanese patients: the large intestine-dominant type has features of intestinal pseudo-obstruction.
Maruyama A,Nagashima T,Iwamoto M,Minota S
Lupus
This study was performed to investigate the clinical characteristics of lupus enteritis in Japanese patients with systemic lupus erythematosus (SLE). A total of 481 patients with SLE admitted to our hospital between 2001 and 2015 were retrospectively reviewed. Diagnosis of lupus enteritis was based on the following three criteria: (1) abdominal symptoms, (2) diffuse long-segment bowel thickening and (3) a requirement for glucocorticoid therapy. Lupus enteritis was identified in 17 patients (3.5%) and there were two distinct types: small intestine-dominant and large intestine-dominant. Significant differences between the two types were noted with respect to the age, frequency of biopsy-proven lupus nephritis, frequency of rectal involvement, maximum bowel wall thickness, and requirement for steroid pulse therapy. Among patients with large intestine-dominant lupus enteritis, 60% had extra-intestinal symptoms (hydroureter, bladder wall thickening, and bile duct dilatation) that are known complications of intestinal pseudo-obstruction. Two patients with large intestine-dominant lupus enteritis developed intestinal pseudo-obstruction either before or after diagnosis of lupus enteritis. Five patients (29%) developed recurrence during a median observation period of 7.2 years (1.4-14.4 years). In conclusion, large intestine-dominant lupus enteritis resembles intestinal pseudo-obstruction and these two diseases may have a common pathogenesis.
10.1177/0961203318785770
Clinical Features, Morbidity, and Risk Factors of Intestinal Pseudo-obstruction in Systemic Lupus Erythematosus: A Retrospective Case-control Study.
Zhang Lingling,Xu Dong,Yang Hong,Tian Xinping,Wang Qian,Hou Yong,Gao Na,Zhang Li,Li Mengtao,Zeng Xiaofeng
The Journal of rheumatology
OBJECTIVE:To analyze the epidemiology, clinical characteristics, and risk factors for systemic lupus erythematosus-related intestinal pseudo-obstruction (SLE-IPO). METHODS:We retrospectively examined 85 patients with SLE with IPO as the case group and 255 randomly matched patients with SLE without any gastrointestinal manifestations as the control group, out of 4331 inpatients at the Peking Union Medical College Hospital (PUMCH) from 2003 to 2014. RESULTS:Over the last 11 years at PUMCH, the prevalence of IPO in patients with SLE was 1.96% and the in-hospital fatality rate was 7.1%. Of these patients, 57.6% presented with IPO as the initial affected system of SLE, and the rate of misdiagnosis was about 78%. Pyeloureterectasis was the most common complication (58.9%) in patients with SLE-IPO and the incidence of biliary tract dilation was 7.1%. Patients with SLE with IPO were always diagnosed at an earlier stage of SLE with a higher frequency of hematological disturbance, polyserositis, and hypocomplementemia. Pyeloureterectasis, hypocomplementemia, and elevated C-reactive protein levels in serum were independent risk factors for IPO in SLE disease. Patients with SLE-IPO with long IPO duration and those diagnosed during late stages of SLE or concurrent with pyeloureterectasis and megacholedochus always had an unfavorable outcome. CONCLUSION:IPO is a rare complication, but commonly presents as the initial affected system of SLE, which can lead to a difficult diagnosis and delayed treatment. SLE-IPO occurrence concomitantly with pyeloureterectasis and megacholedochus showed a severe clinical situation in our cohort. Thus, patients with SLE-IPO with systemic smooth muscular involvement should be diagnosed early and treated aggressively.
10.3899/jrheum.150074
Major gastrointestinal manifestations in lupus patients in Asia: lupus enteritis, intestinal pseudo-obstruction, and protein-losing gastroenteropathy.
Chng H H,Tan B E,Teh C L,Lian T Y
Lupus
Gastrointestinal (GI) symptoms are common in patients with systemic lupus erythematosus (SLE) and may be due to the disease itself, side-effects of medications, or non-SLE causes. However, GI manifestations of lupus attract far less attention than the other major organ involvements, are infrequently reviewed and rarely documented in published lupus databases or cohort studies including those from countries in Asia. According to three reports from two countries in Asia, the cumulative prevalence of SLE GI manifestations range from 3.8% to 18%. In this review, we focus on three major GI manifestations in patients from Asian countries: lupus enteritis, intestinal pseudo-obstruction, and protein-losing gastroenteropathy, for which early recognition improves outcome and reduces morbidity and mortality.
10.1177/0961203310374337
Gastrointestinal and hepatic involvement in paediatric systemic lupus erythematosus.
Clinical and experimental rheumatology
Systemic lupus erythematosus (SLE) is a multisystemic, autoimmune, inflammatory disease. Gastrointestinal (GI) involvement, extensively described in adults, is less characterised in paediatric-onset SLE (pSLE). The aim of the present narrative review was to provide a comprehensive summary and update on GI involvement in pSLE. A literature search on PubMed and EMBASE was conducted to identify original articles, reviews, case series and editorials published in English from 2000 to 31 August 2020. Based on this, we reported the prevalence, pathogenetic mechanisms, clinical issues, diagnostic tools and management of each form of GI involvement in pSLE. Lupus enteritis is the most frequent type of GI involvement in pSLE, followed by intestinal pseudo-obstruction, protein-losing enteropathy, hepatic disease and acute pancreatitis. The most common presenting GI symptoms are non-specific and include abdominal pain, anorexia, nausea, vomiting. In most cases, they are associated with other clinical and laboratory manifestations of SLE. The complications of GI involvement, including perforation and intestinal infarction, can be life-threatening. Laboratory findings and imaging studies can help to rule out non-SLE related causes for GI manifestations and to reveal typical features of the single forms of GI involvement. Early diagnosis and treatment are crucial to improve prognosis and avoid unnecessary surgery. Most SLE GI manifestations respond well to glucocorticoids and immunosuppressants. In conclusion, GI involvement is frequent in pSLE and its diagnosis and management can be a challenge for clinicians. In view of the limited available data, further studies are needed to better explore the prevalence, prognosis and treatment recommendations for GI involvement in pSLE.
10.55563/clinexprheumatol/oebrcq
Systemic lupus erythematosus simultaneously presenting with visceral muscle dysmotility syndrome and mechanical intestinal obstruction clinically relieved by surgery: a case report and literature review.
Wen Junxian,Chen Weijie,Gao Lu,Qiu Xiaoyuan,Lin Guole
BMC gastroenterology
BACKGROUND:Intestinal pseudo-obstruction (IPO) accompanied by hepatobiliary dilatation and ureterohydronephrosis is extremely rare in systemic lupus erythematosus (SLE). This triad is also called visceral muscle dysmotility syndrome (VMDS). Only 9 cases have been reported in the literature. Here, we report a rare case of VMDS with mechanical intestinal obstruction that was clinically relieved by surgery. CASE PRESENTATION:This report refers to a 31-year-old woman with SLE and gastrointestinal symptoms presented as abdominal pain, nausea and stoppage of the passage of flatus or stool without obvious reasons. The patient suffered from severe abdominal distension because of massive flatulence. Contrast-enhanced computed tomography (CT) of the abdomen performed in our hospital showed localized stenosis of the bowel, ureterohydronephrosis, and biliary tract dilatation. Endoscopy showed a stenotic segment located in the sigmoid colon. The colon biopsy samples suggested that the stenosis was caused by inflammatory tissues. Biochemical investigations showed hypoalbuminemia, electrolyte disturbance and decreased C3. Antinuclear antibody was positive. After careful assessment, transverse colostomy was performed for this patient. Gastrointestinal symptoms were clinically relieved after the surgery. CONCLUSION:To the best of our knowledge, no VMDS patients have presented with mechanical ileus before. This case is the first documented occurrence of SLE with VMDS and mechanical intestinal obstruction symptoms relieved by surgery. Due to the low incidence of this condition, no standard treatment regimen has been established. However, surgical treatment offers significant benefit in specific situations.
10.1186/s12876-022-02105-3
Intestinal pseudo-obstruction and ureterohydronephrosis as the presenting manifestations of relapse in a lupus patient.
Alexopoulou A,Andrianakos A,Dourakis S P
Lupus
Intestinal pseudo-obstruction (IPO) is a rare complication of systemic lupus erythematosus (SLE). We present a 32-year old female with SLE for seven years. She was admitted with profound fatigue, frequent vomiting, colicky abdominal pain, diarrhoea and intermittent dysuria for the past 12 months. Imaging studies revealed dilated small and large bowel loops with thickened intestinal wall and multiple fluid levels. Urinary tract involvement was also demonstrated. The patient responded well to immunosuppressive treatment. IPO in the context of SLE has been described only in anecdotal case reports. Half of the cases developed this complication during the course of lupus as in the present case. Concomitant ureterohydronephrosis was present in approximately two-thirds of the cases. Early recognition of the syndrome is necessary for the institution of the appropriate medical treatment and prevention of inappropriate surgical intervention.
10.1191/0961203304u1064cr
Generalized megaviscera of lupus: refractory intestinal pseudo-obstruction, ureterohydronephrosis and megacholedochus.
Park Frederick-D,Lee Jeffrey-K,Madduri Ganga-D,Ghosh Pradipta
World journal of gastroenterology
Dilated dysfunction involving multiple visceral organs has been reported in patients with systemic lupus erythematosus (SLE). Chronic intestinal pseudo-obstruction (CIPO) resulting from intestinal smooth muscle damage has presented in conjunction with ureterohydronephrosis and, more rarely, biliary dilatation (megacholedochus). While the molecular pathogenesis is largely unknown, observed histopathologic features include widespread myositis, myocyte necrosis in the intestinal muscularis propria with subsequent atrophy and fibrosis, preserved myenteric innervations and little vasculitis. High dose immunosuppression usually results in resolution of symptoms with recovery of smooth muscle function, indicative of an autoimmune etiology. We report a patient with SLE who presented with intestinal pseudo-obstruction, ureterohydronephrosis and megacholedochus, and present images that illustrate megaviscera simultaneously involving all 3 visceral organs. Since the co-manifestation of all 3 is unusual and has been reported only once previously, we have termed this rare clinical syndrome generalized megaviscera of lupus (GML). Although the SLE disease-activity parameters responded to aggressive immunomodulative therapy in our patient, clinical evidence of peristaltic dysfunction persisted in all involved viscera. This is a variation from the favorable outcomes reported previously in SLE patients with GML and we attribute this poor clinical outcome to disease severity and, most importantly, delayed clinical presentation. Since inflammation followed by atrophy and fibrosis are key aspects in the pathogenesis and natural history of GML, the poor response in our patient who presented late in the clinical course may be the result of 'burnt out' inflammation with irreversible end-stage fibrosis. Thus, early recognition and timely initiation of treatment may be the key to recover visceral peristaltic function in patients with GML.
10.3748/wjg.15.3555
Clinical analysis of 61 systemic lupus erythematosus patients with intestinal pseudo-obstruction and/or ureterohydronephrosis: a retrospective observational study.
Xu Na,Zhao Jiuliang,Liu Jinjing,Wu Di,Zhao Lidan,Wang Qian,Hou Yong,Li Mengtao,Zhang Wen,Zeng Xuejun,Fang Weigang,Huang Xiaoming,Zhang Xuan,Tian Xinping,Zhao Yan,Zeng Xiaofeng,Zhang Fengchun
Medicine
The objective of this article is to investigate the clinical features of intestinal pseudo-obstruction (IPO) and/or ureterohydronephrosis in systemic lupus erythematosus (SLE). Sixty-one SLE patients with IPO and/or ureterohydronephrosis were analyzed retrospectively. A total of 183 cases were randomly selected as controls from 3840 SLE inpatients without IPO and ureterohydronephrosis during the same period. Patients were assigned to 1 of the 3 groups (SLE with IPO and ureterohydronephrosis, SLE with IPO, and SLE with ureterohydronephrosis). The clinical characteristics, treatments, and prognosis were compared between the 3 groups. There were 57 females and 4 males, with a mean age of 32.0 years. IPO was the initial manifestation of SLE in 49.1% of the cases, whereas ureterohydronephrosis in 32.5%. All patients were initially treated with a high-dose steroid. Thirty-one of these patients (50.8%) also received intravenous methylprednisolone pulse therapy. Two patients died of bowel perforation and lupus encephalopathy, and the other 59 patients (96.7%) achieved remission after treatment. The incidences of fever, glomerulonephritis, nervous system involvement, serositis, erythrocyte sedimentation rate elevation, hypoalbuminemia, hypocomplementemia, and anti-SSA antibody positivity were significantly higher in patients with IPO and/or ureterohydronephrosis than in the control group (without IPO and ureterohydronephrosis). Also, patients with IPO and/or ureterohydronephrosis had higher SLE Disease Activity Index scores than control patients. Compared with SLE patients with IPO, the patients with IPO and ureterohydronephrosis had a significantly higher incidence of gallbladder wall thickening, biliary tract dilatation, and serositis, whereas the patients with ureterohydronephrosis had less mucocutaneous involvement and serositis. Eight of the 47 IPO patients who initially responded well to immunotherapy relapsed; however, all responded well to retreatment with adequate immunotherapy. Of these 8 patients, 4 relapsed following poor compliance and self-discontinuation of steroid or immunosuppressant therapy. The rate of poor compliance with immunotherapy and the number of organ systems involved in patients in the recurrent IPO group were significantly higher than those in the nonrecurrent IPO group. IPO and ureterohydronephrosis are severe complications of SLE. As patients usually respond readily to early optimal steroid treatment, early diagnosis and timely initiation of glucocorticoid are important to relieve symptoms, prevent complications, and improve prognosis.
10.1097/MD.0000000000000419
Gastrointestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment.
Tian Xin-Ping,Zhang Xuan
World journal of gastroenterology
Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by SLE. Gastrointestinal symptoms are common in SLE patients, and more than half of them are caused by adverse reactions to medications and viral or bacterial infections. Though not as common as lupus nephritis, SLE-related gastrointestinal involvement is clinically important because most cases can be life-threatening if not treated promptly. Lupus mesenteric vasculitis is the most common cause, followed by protein-losing enteropathy, intestinal pseudo-obstruction, acute pancreatitis and other rare complications such as celiac disease, inflammatory bowel diseases, etc. No specific autoantibody is identified as being associated with SLE-related gastroenteropathy. Imaging studies, particularly abdominal computed tomography scans, are helpful in diagnosing some SLE-related gastroenteropathies. Most of these complications have good therapeutic responses to corticosteroids and immunosuppressive agents. Supportive measures such as bowel rest, nutritional support, antibiotics and prokinetic medications are helpful in facilitating functional recovery and improving the outcome.
10.3748/wjg.v16.i24.2971
Intestinal Pseudo-Obstruction as the Initial Clinical Presentation in Systemic Lupus Erythematosus: A Rare and Severe Disorder.
Nyabera Akwe,Elfishawi Mohanad,Cuevas Francisco,Riaz Fahad,Abrudescu Adriana
Case reports in gastrointestinal medicine
Intestinal pseudo-obstruction (IPO) is a rarely recognized complication of systemic lupus erythematosus (SLE). We present a 36-year-old African American female, with only known past medical history of anemia, admitted for frequent vomiting, abdominal distension, abdominal pain, diarrhea, and fever that had been ongoing for 5 days. Laboratory results revealed leukopenia and thrombocytopenia. Imaging revealed dilated small bowel loops, abdominal ascites, as well as mild bilateral hydroureteronephrosis without obstructing calculus. Serologic testing confirmed a diagnosis of SLE. The patient was placed on immunosuppressive therapy and responded well. IPO has previously been described as a rare finding in patients with SLE, with bilateral hydroureteronephrosis and lupus interstitial cystitis having been noted as common concomitant factors. One must have a high level of suspicion to recognize it as being one of the initial clinical presentations. Early recognition and appropriate management preclude unnecessary invasive procedures that do not take into account the pathophysiology of the condition and allow for appropriate management and return of peristaltic function.
10.1155/2020/8873917
Chronic intestinal pseudo-obstruction in patients with systemic lupus erythematosus: report of four cases.
Ceccato Federico,Salas Adrián,Góngora Vanina,Ruta Santiago,Roverano Susana,Marcos Juan Carlos,Garcìa Mercedes,Paira Sergio
Clinical rheumatology
Chronic intestinal pseudo-obstruction (CIPO), a recently recognized manifestation of systemic lupus erythematosus (SLE) with only 23 cases reported in the English literature, may appear as a complication or as the initial presentation of SLE and usually occurs during the setting of an active lupus. The pathogenic mechanism in SLE is unknown. We describe four additional cases with clinical, radiological, and manometric features of CIPO. As SLE-related CIPO usually responds to treatment with high doses of corticosteroids and/or immunosuppressive and prokinetic agents, a high level of awareness of this complication is needed to avoid unnecessary surgical intervention.
10.1007/s10067-007-0760-5
Intestinal pseudo-obstruction associated with biliary tract dilatation in a patient with systemic lupus erythematosus.
Kansal A,Jain A,Thenozhi S,Agarwal V
Lupus
Intestinal pseudo-obstruction (IpsO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a patient with SLE who presented with IpsO and biliary dilatation (megacholedochus). The co-manifestation of these two conditions in SLE is unusual and has been reported only twice previously. The patient responded well to immunosuppressive treatment. IpsO is a rare but recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IpsO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.
10.1177/0961203312464091
An Atypical Initial Manifestation of Systemic Lupus Erythematosus: Lupus Enteritis Accompanied by Intestinal Pseudo-Obstruction and Bilateral Hydronephroureter.
Cureus
Systemic lupus erythematosus (SLE) is a systemic, autoimmune, multisystem disease. Lupus enteritis accompanied by intestinal pseudo-obstruction (IPO) is a serious and rare initial manifestation that can lead to high mortality and morbidity in case of delay in diagnosis and treatment. Here, we present a very complicated case of a 36-year-old female Pakistani patient with lupus enteritis accompanied by IPO and bilateral hydronephroureter. The patient had a three-month history of fever, weight loss, recurrent diarrhea, vomiting, alopecia, and photosensitivity. She had a malar and discoid rash, with signs and symptoms of IPO and neuropsychiatric lupus. Her labs revealed positive anti-nucleosome antibodies (8 U/mL), anti-Ro antibodies (100 U/mL), and anti-La antibodies (53 U/mL); equivocal anti-dsDNA antibodies (7 U/mL) and anti-Sm antibodies (7 U/mL); direct Coomb's positive hemolytic anemia; raised C-reactive protein and erythrocyte sedimentation rate levels; low complement (C3 and C4) levels; and pyuria. IPO was evident on abdominal X-ray and CT scan. Her Systemic Lupus Erythematosus Disease Activity Index was 24, indicating severe disease flare. She was treated with intravenous methylprednisolone, hydroxychloroquine, and intravenous 500 mg cyclophosphamide. Her lab parameters and clinical mini-mental score improved, from 0/30 to 18/30. She was discharged on oral prednisolone 0.5 mg/kg/day, hydroxychloroquine, trimethoprim-sulfamethoxazole (prophylaxis for pneumonia), and mineral and vitamin supplements. She was followed up on the 15th day of discharge for the next dose of cyclophosphamide, and her clinical and lab parameters were normal at that time with gradual improvement in cognition. Lupus enteritis with coexisting IPO and bilateral hydronephroureter poses a diagnostic and therapeutic challenge because of atypical and uncommon manifestations of lupus and overlapping features with intestinal tuberculosis and other inflammatory bowel conditions.
10.7759/cureus.50628
[Chronic intestinal pseudo-obstruction in systemic lupus erythematosus].
Ghannouchi Jaafoura N,Khalifa M,Atig A,Ben Jazia E,Alaoua A,Braham Krifa A,Letaief A,Bahri F
La Revue de medecine interne
Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.
10.1016/j.revmed.2010.03.458
An Uncommon Manifestation of Systemic Lupus Erythematosus as Lupus Enteritis With Intestinal Pseudo-Obstruction and Invasive Candidiasis.
Cureus
Systemic lupus erythematosus (SLE) is a multifaceted autoimmune disorder, occasionally presenting with rare complications like lupus enteritis (LE) and intestinal pseudo-obstruction (IPO). We present a unique case of a 32-year-old woman with LE and IPO, complicated by invasive candidiasis, as an initial manifestation of SLE. The patient presented with a 15-day history of abdominal pain, vomiting, and poor oral intake, and was initially misdiagnosed with infective enterocolitis. Examination revealed abdominal distension and absent bowel sounds due to IPO, alongside severe hypokalemia and signs of intra-abdominal hypertension (IAH), necessitating ventilator support. Subsequent workup confirmed SLE with LE and associated lupus nephritis (LN). The patient's condition was further complicated by disseminated invasive candidiasis involving multiple organs, including the bloodstream, chorioretinitis, and endocarditis. Despite her critical state, intensive multidisciplinary care, including high-dose steroids, antifungal therapy, and supportive measures, led to her recovery and discharge after a 51-day ICU stay. This case underscores the complexity of diagnosing SLE when it presents with non-specific symptoms. The concomitant occurrence of LE, IPO, and invasive candidiasis is particularly rare, highlighting the need for high clinical suspicion in the presence of SLE serological activity. The presence of invasive candidiasis was likely secondary to gut translocation due to LE-associated inflammation, a phenomenon not previously well-documented. LE can manifest as the primary and sole presentation of SLE, even in the absence of typical lupus features. Prompt immunomodulatory treatment and comprehensive care are essential for a favorable outcome. Clinicians should consider invasive candidiasis in SLE patients with acute GI involvement, particularly in the presence of LE.
10.7759/cureus.70485
Pseudo-pseudo Meigs syndrome in systemic lupus erythematosus misdiagnosed as pseudo-Meigs' syndrome: A case report.
The journal of obstetrics and gynaecology research
Symptoms of pelvic masses, elevated serum CA125 levels, massive ascites, and pleural effusion in female patients are usually associated with malignancy. Some benign ovarian tumors or other nonmalignant tumors may also produce similar symptoms, called Meigs syndrome or pseudo-Meigs' syndrome, which should be one of the differential diagnoses. However, there is an extremely rare form of SLE called pseudo-pseudo Meigs syndrome (PPMS), which may also present with the above symptoms, but is not associated with any of the tumors. In this paper, we report a case of a 47-year-old woman who presented with abdominal distention. The patient was found to have elevated serum CA125 levels to 182.9 U/mL before the operation. Her PET-CT suggested a large heterogeneous mass in the pelvis measuring 8.2 × 5.8 cm with a large amount of ascites. She was initially diagnosed with ovarian cancer and underwent exploratory laparotomy. Pathology of the surgical specimen revealed a uterine leiomyoma. Two months after discharge, the patient's ascites reappeared along with recurrent intestinal obstruction. After ascites and serological tests, she was eventually diagnosed with systemic lupus erythematosus and received systemic hormonal therapy.
10.1111/jog.15677
Pseudo-pseudo Meigs' syndrome (PPMS) in chronic lupus peritonitis: a case report with review of literature.
Meena Durga Shankar,Kumar Bharat,Gopalakrishnan Maya,Kachhwaha Arjun,Kumar Saurabh,Sureka Binit,Gupta Shruti,Bohra Gopal Krishana,Garg Mahendra Kumar
Modern rheumatology case reports
Gastrointestinal involvement in systemic lupus erythematosus (SLE) usually occurs in the form of mesenteric vasculitis, protein-losing enteropathy, intestinal pseudo-obstruction, and pancreatitis. We describe a 23-year-old female, a known case of SLE presented with significant ascites and pleural effusion. Further evaluation showed elevated CA-125 levels without evidence of malignancy. The patient was treated with corticosteroids, hydroxychloroquine, and azathioprine resulting in the resolution of ascites in 2 weeks. The triad of ascites, pleural effusion, and increased CA-125 is known as pseudo-pseudo Meigs' syndrome, which is rarely reported in the literature. Clinicians should be aware of this entity while evaluating an SLE patient with low serum-ascites albumin gradient (SAAG) ascites.
10.1080/24725625.2021.1916160
A Flare-up of Systemic Lupus Erythematosus with Unusual Enteric Predominance.
Ronen Joshua A,Mekala Armugam,Wiechmann Catherine,Mungara Sai
Cureus
Enteritis associated with systemic lupus erythematosus (SLE) is a rare and unusual manifestation of the gastrointestinal (GI) consequences of SLE itself. Complications of the enteritis component include mesenteric vasculitis, intestinal pseudo-obstruction, and protein-losing enteropathy. Lupus enteritis is very responsive to treatment with pulse steroids in almost 70% of the patients, but it is critical to diagnose it early to prevent devastating organ damage. The case describes a 21-year-old Caucasian female with a past medical history of uncomplicated laparoscopic appendectomy (one month prior to the time of presentation), major depressive disorder, asthma, iron deficiency anemia, pelvic inflammatory disease secondary to sexually transmitted Chlamydia trachomatis infection, and SLE (diagnosed two weeks prior to presentation). She had been transferred from an outside facility with complaints of severe right upper quadrant (RUQ) abdominal pain for one day. The patient had run out of her prescription for steroids and hydroxychloroquine two days prior to the presentation. Her abdominal pain was accompanied by nausea, bilious vomiting, non-bloody diarrhea, a photosensitive facial rash, left-sided pressure-type periorbital headache, diplopia, oral ulcers, inappetence, joint stiffness, and muscle weakness. A CT of the abdomen and pelvis from an outside facility showed enteritis involving the proximal jejunum with associated mesenteric edema and ascites, suggesting infectious versus inflammatory or autoimmune etiology. A repeat CT scan a few days later confirmed these findings along with adjacent mesenteric fat stranding. Her autoimmune workup confirmed the serological diagnosis of SLE, and assessment of the SLE Disease Activity Index (SLEDAI) confirmed the diagnosis of a severe SLE flare. Upper endoscopy detected edematous mucosa in the duodenum and jejunum without active bleeding, gastropathy, or ulceration. No surgical intervention was required. Her symptoms resolved with supportive care, pulse steroids, and hydroxychloroquine. She was discharged with instructions for outpatient follow-up with gastroenterology and rheumatology.
10.7759/cureus.7068
Visceral muscle dysmotility syndrome in systemic lupus erythematosus: case report and review of the literature.
Chen Yu Qiang,Xue Qing,Wang Nian Song
Rheumatology international
Intestinal pseudo-obstruction (IPO) is not uncommon in systemic lupus erythematosus (SLE), and IPO in SLE has an apparent association with ureterohydronephrosis. However, hepatobiliary dilatation without mechanical obstruction presenting together with IPO and ureterohydronephrosis is much more scarce in SLE. Here, we named this rare triad of IPO, ureterohydronephrosis, and biliary tract dilatation as visceral muscle dysmotility syndrome (VMDS). It always imitates an acute abdomen and is even life-threatening if treated incorrectly. To diagnose a VMDS, infections and mechanical obstructions should be ruled out carefully. Here, we report a 24-year-old Chinese woman with SLE who presented of VMDS that associated with corticoids tapering induced SLE flare. In this case, early vigorous immunosuppressive treatment conquered the triad timely and thus yielded a good outcome.
10.1007/s00296-011-1856-4
Clinical analysis of systemic lupus erythematosus with gastrointestinal manifestations.
Xu D,Yang H,Lai C-C,Li P,Zhang X,Yang X-O,Zhang F-C,Qian J-M
Lupus
Our objective was to analyse the clinical characteristics of systemic lupus erythematosus (SLE) patients with gastrointestinal manifestations. Medical charts of 177 hospitalized SLE patients were systematically reviewed, including demographic data, clinical features, laboratory findings, and treatments, as well as outcomes. Thirty-nine cases (22.0%) had SLE-related gastrointestinal manifestations, and in 12 cases (30.8%), gastrointestinal manifestations occurred as the initial symptoms. Twenty-five cases (64.1%) had abdominal pain, 22 cases (56.4%) had nausea and vomiting, 12 cases (30.8%) had diarrhea, and gastrointestinal hemorrhage occurred in three cases (7.7%). Protein losing enteropathy and intestinal pseudo-obstruction were the most common identifiable gastrointestinal complications, though other reasons such as superior mesenteric venous thrombosis, pancreatitis, peritonitis, and liver impairment could also occur in SLE. The incidences of Raynaud's phenomenon and pyeloureterectasis were significantly higher in patients with gastrointestinal complications than those without (p < 0.05). Multivariable analysis indicated Raynaud's phenomenon, decreased C3, CH50, and anti-neutrophil cytoplasmic antibody positivity were independent predictors of gastrointestinal involvements (p < 0.05). Gastrointestinal complications are common, diverse, and could be the initial and major manifestations of lupus. SLE patients who had Raynaud's phenomenon, hypocomplementemia and positive anti-neutrophil cytoplasmic antibody were at increasing risk of developing gastrointestinal complication.
10.1177/0961203310365883
Systematic review of treatments for the gastrointestinal manifestations of systemic lupus erythematosus.
Seminars in arthritis and rheumatism
OBJECTIVES:To comprehensively assess and present the evidence for treatments used in the management of the gastrointestinal manifestations of SLE. METHODS:A systematic search of the literature from January 1990 to June 2022 was performed using the following databases: MEDLINE, EMBASE, PubMed and Cochrane. Key words relating to the gastrointestinal system, SLE, and treatment were used. Where there was sufficient evidence for the treatment of a manifestation, we excluded case series with <10 cases and case reports. However, for rarer manifestations with insufficient higher-level evidence, smaller case series and case reports were included. RESULTS:A total of 29 studies including 767 patients were included in the review; six cohort studies, 11 case-control studies, and 11 case series. Specific gastrointestinal manifestations included enteritis (5 studies), mesenteric vasculitis (3 studies), acute pancreatitis (5 studies), chronic pancreatitis (1 study), intestinal pseudo-obstruction (IPO) (2 studies), hepatitis (4 studies), protein-losing enteropathy (PLE) (6 studies), acute acalculous cholecystitis (2 studies), and Budd-Chiari Syndrome (1 study). Evidence for the treatment of Ascites (13 case reports), peritonitis (3 case reports), and miscellaneous GI manifestations (11 case reports) are included as a supplemental file. Most studies demonstrated a benefit from pulsed intravenous methylprednisolone (IVMP) in severe or life-threatening manifestations, and oral prednisolone for less severe manifestations. However, the quality of evidence was low, with a high risk of bias in all studies. CONCLUSION:This review highlights the need for standardised disease definitions and terminology, as well as consideration of including gastrointestinal manifestations in disease scoring systems. There is a significant need for high-quality clinical trials in the treatment of the gastrointestinal manifestations of SLE, which will likely need to be multi-centre. We hope that this review will promote awareness of the gastrointestinal manifestations of SLE, and serve as a practical guide for evidence-based treatment.
10.1016/j.semarthrit.2024.152567
Autoimmune Lupus Enteritis with Pan Gastrointestinal Involvement in an Adult Patient with Systemic Lupus Erythematosus: Complete Response to Belimumab.
Journal of community hospital internal medicine perspectives
Autoimmune lupus enteritis in systemic lupus erythematosus (SLE) is a rare manifestation that comprises of gastrointestinal tract inflammation with supportive images and/or biopsy findings. We report a unique case of widespread lupus enteritis occurring late in the disease process and in isolation without additional features of active SLE but in the presence of SLE serological activity. There was no clear evidence of active mesenteric vasculitis, intestinal pseudo-obstruction, protein-losing enteropathy, or coagulopathy by imaging or histopathology. This is the first reported case of an SLE patient with pan-gastrointestinal involvement of lupus enteritis responding to Belimumab, with complete resolution of the gastrointestinal syndrome and no further recurrence of gastrointestinal events. Rapid diagnosis and prompt immunomodulatory treatment of lupus-related enteritis are critical to avoid potentially life-threatening complications.
10.55729/2000-9666.1185
Clinical features and associated factors of abdominal pain in systemic lupus erythematosus.
Yuan Shiwen,Lian Fan,Chen Dongying,Li Hao,Qiu Qian,Zhan Zhongping,Ye Yujin,Xu Hanshi,Liang Liuqin,Yang Xiuyan
The Journal of rheumatology
OBJECTIVE:To evaluate the clinical characteristics of systemic lupus erythematosus (SLE)-induced abdominal pain in a cohort in South China and identify the risk factors for SLE-induced abdominal pain. METHODS:This is a retrospective cohort study of SLE patients with complaint of abdominal pain admitted to the first affiliated university hospital of Sun Yat-sen University between 2002 and 2011. Demographic information, clinical features, laboratory findings, SLE Disease Activity Index, and imaging characteristics were documented. RESULTS:Of the 3823 SLE patients reviewed, 213 patients complained of abdominal pain and 132 cases were considered SLE-induced. The most common causes were lupus mesenteric vasculitis (LMV; 73.5%, 97/132) and lupus pancreatitis (LP; 17.4%, 23/132). Other causes included appendicitis, acute gastroenteritis, and peritonitis. Univariate and multivariate logistic regression analysis indicated the European Consensus Lupus Activity Measurement (ECLAM) score was significantly associated with lupus-induced abdominal pain (OR = 1.858, 95% CI: 1.441-2.394, p < 0.001), LMV (OR = 1.713, 95% CI: 1.308-2.244, p < 0.001), and LP (OR = 2.153, 95% CI: 1.282, 3.617, p = 0.004). The serum D-dimer level (OR = 1.004, 95% CI: 1.002-1.005, p < 0.001) was a strongly associated factor for lupus-induced abdominal pain. Moderate and large amounts of ascetic fluid was significantly associated with lupus-induced abdominal pain and LMV. Elevated liver enzymes was a risk factor for LP (OR = 34.605, 95% CI: 3.591-333.472, p = 0.002). CONCLUSION:LMV and LP were the leading causes of SLE-induced abdominal pain. The serum D-dimer was a strongly associated factor for lupus-induced abdominal pain. ECLAM score was a reliable index in assessment of SLE-associated abdominal pain. Elevated liver enzymes, and moderate or large amounts of ascites, were positively associated with lupus-induced abdominal pain.
10.3899/jrheum.130492
Acute pan-dysautonomia as well as central nervous system involvement and peripheral neuropathies in a patient with systemic lupus erythematosus.
Yukawa Sonosuke,Tahara Koichiro,Shoji Aki,Hayashi Haeru,Tsuboi Norioki
Modern rheumatology
A 32-year-old woman was diagnosed with leucopenia in 2002, being antinuclear antibody, anti-DNA antibody, and antiphospholipid antibody positive, and she was administered low-dose aspirin. In July 2006, she was admitted to our hospital because of pyrexia and abdominal pain. Examination revealed paralytic ileus, absence of the pupillary light reflex, dyshidrosis and anuresis. In addition, with high-level interleukin-6 in cerebrospinal fluid, the sensory nerve conduction velocity was derivation impotence. She was subsequently diagnosed with systemic lupus erythematosus (SLE) with central nervous system involvement, peripheral neuropathy as well as acute pan-dysautonomia. After pulse corticosteroid therapy, paralytic ileus was improved, however, the urination disorder persisted, and syncope due to orthostatic hypotension became marked. Plasma exchange and a second course of pulse corticosteroid therapy were performed, and were ineffective, whereas intravenous cyclophosphamide was effective. This patient is a rare case of central nervous system, peripheral neuropathy as well as acute pan-dysautonomia with SLE.
10.1007/s10165-008-0087-3
Is there an ethnic difference in the prevalence of lupus cystitis? A report of six cases.
Chen Mao-Yuan,Lee Kuang-Lun,Hsu Ping-Ning,Wu Chien-Sheng,Wu Cheng-Han
Lupus
Lupus cystitis was rare but frequently resulted in obstructive uropathy and had a strong association with gastrointestinal (GI) symptoms. We treated six patients with systemic lupus erythematosus (SLE) and obstructive uropathy from January 1996 to December 2001 in a university hospital. Evidence of cystitis was obtained from cystoscopic biopsy or the presence of thickened bladder wall in image study. Similar to other reports, five patients had GI manifestations such as abdominal pain, nausea/vomiting, diarrhoea or ileus. In addition, mesenteric lymphadenopathy or pancreatitis was noted in three patients. Two patients had been treated for idiopathic thrombocytopenic purpura (ITP), four and 20 years ago, respectively. All six patients had antibodies to double-stranded DNA (dsDNA). Five patients each had antibodies to cardiolipin (IgG aCL) or SSA. The high prevalence of anti-SSA had also been reported in Chinese lupus patients with intestinal pseudo-obstruction, a clinical manifestation frequently associated with bilateral ureterohydronephrosis. Two patients died of intractable infection after the surgical procedures for persistent ureterohydronephrosis and both patients had antibodies to ribosomal P proteins. Lupus cystitis might not be so rare in Chinese patients with SLE. The diagnosis should be kept in mind when lupus patients have urinary and/or GI symptoms.
10.1191/0961203304lu527cr
Clinical observation pseudoobstruction syndrome of the stomach's output part and small intestine of a patient with systematic lupus erythematosis.
Gilyarov M Yu,Shostak N A,Kotovа D P,Schekochikhin D Yu,Kasha Yu O
Terapevticheskii arkhiv
Stomach's output part and small intestine, combining with damaging of the urinary tract is a rare systemic lupus eritematosus (SLE) manifestation. The patient is 32 years old, suffering from SLE with damaged join, blood system, secondary antiphospholipid syndrome with pulmonary embolism in the history and formation of high postembolic pulmonary hypertension on therapy with hydroxychloroquine and low doses of corticosteroids, was hospitalized because of persistent nausea, vomiting, loss of more than 10 kg body weight 1.5 months. The research have shown the obstruction's formation of the stomach's output part, small bowel obstruction at several levels, as well as thickening of the bladder wall and the unilateral expansion of the ureter. Against the backdrop of strengthening of immunosuppressive therapy these lesions completely regressed.
10.26442/terarkh201890298-101
Chronic intestinal pseudoobstruction associated with autoantibodies against proliferating cell nuclear antigen.
Nojima Y,Mimura T,Hamasaki K,Furuya H,Tanaka G,Nakajima A,Matsuhashi N,Yazaki Y
Arthritis and rheumatism
Autoantibodies against proliferating cell nuclear antigen (PCNA) have been detected almost exclusively in sera from patients with systemic lupus erythematosus (SLE). Because of the extremely low prevalence of anti-PCNA, however, it has been difficult to establish its disease specificity and clinical associations. We present 2 cases in which patients with anti-PCNA developed severe gastrointestinal dysfunction that led to chronic intestinal pseudoobstruction. Neither patient had manifestations suggestive of SLE. Although sclerodermatous skin changes were minimal or absent in these patients, physical and radiographic findings indicated that systemic sclerosis was a likely cause of the gastrointestinal dysfunction. The similarity of the clinical features in these patients implies that the unusual detection of this unique autoantibody may not be a coincidence.
10.1002/art.1780390522
Gastrointestinal manifestations of systemic lupus erythematosus.
Luman W,Chua K B,Cheong W K,Ng H S
Singapore medical journal
Gastrointestinal symptoms are common in patients with systemic lupus erythematosus (SLE) and can be due to primary gastrointestinal disorders, complications of therapy or SLE itself. In this case report, we describe three different presentations and causes of gastrointestinal complaints in patients with SLE. Diagnostic and management problems are discussed.
Lupus cystitis and performation of the small bowel in a patient with systemic lupus erythematosus and overlapping syndrome.
Moriuchi J,Ichikawa Y,Takaya M,Shimizu H,Tokunaga M,Eguchi T,Izumi M,Ohta W,Katsuoka Y,Nakajima I
Clinical and experimental rheumatology
We describe a patient with systemic lupus erythematosus (SLE) and overlapping syndrome who had repeated gastrointestinal (GI) symptoms such as nausea, vomiting and malabsorption. With a subacute process, she developed paralytic ileus and contracted bladder, and died of perforation of the ileum. When 13 reported cases with lupus cystitis were reviewed, an extremely strong correlation between GI and urinary tract symptoms was observed, indicating the presence of a unique subgroup of SLE.