Laboratory features and pharmacological management of early and late-onset primary Sjögren's syndrome.
Rheumatology international
BACKGROUND:Primary Sjögren's Syndrome (pSS) is a systemic chronic autoimmune disorder that contributes to dry mouth (xerostomia) and eyes (xerophthalmia). It mainly affects females between 40 and 60 years old. So far, there is no treatment to cure SS; however, there is a list of medications that can ameliorate the symptoms. In addition, there has been no single test until now to detect pSS, but clinical and immunological investigations are applied as diagnostic tools. Therefore, this study aimed to explore the characteristics of pSS in Saudi patients based on the onset of the disease through laboratory findings and pharmaceutical management. METHODOLOGY:This retrospective study examined diagnosed patients with pSS between 2018 and 2023 from the National Guard Hospital, Saudi Arabia. Data of pSS patients was categorized into two groups: early (under 40 years old) and late-onset (40 years old and above). Data on demographic information, mortality rate, and blood tests such as complete blood count (CBC), creatinine, erythrocyte sedimentation rate (ESR), and vitamin levels, in addition to prescribed medications, were collected from the patient's medical record. Chi-square and t-tests were mainly used, and statistical significance was determined at a P-value less than 0.05. RESULTS:A total of 453 patients were included in the study, where the early-onset group comprised 136 and the late-onset group comprised 317 patients. The mean age of the early and late onset was 34.2 and 60.4, respectively. ESR was significantly higher in the early (46.3 mm/hr) and late-onset (49.8 mm/hr). The most common medication used by all pSS patients was hydroxychloroquine. However, artificial tears were mainly observed in the late-onset group. Other medications, such as pilocarpine, methotrexate, and azathioprine, were prescribed to pSS patients to a lesser extent. CONCLUSION:This study suggests that the onset of pSS could occur even before the age of 40 among Saudi citizens. Notably, elevated ESR levels appeared to be a feature of pSS, which was consistent with other previous findings. The variability of some medications between early-onset and late-onset pSS may indicate disease progression. However, further investigations are required to confirm this observation.
10.1007/s00296-024-05626-0
Differences in clinical phenotypes of primary Sjögren's syndrome depending on early or late onset.
Sebastian Agata,Madej Marta,Sebastian Maciej,Morgiel Ewa,Wawryka Piotr,Wiland Piotr
Advances in clinical and experimental medicine : official organ Wroclaw Medical University
BACKGROUND:Previous research suggests that systemic involvement in primary Sjögren's syndrome (pSS) is a marker of disease prognosis. OBJECTIVES:To evaluate pSS disease activity and the clinical phenotype of pSS patients depending on the age at diagnosis with long-term follow-up. MATERIAL AND METHODS:The study group consisted of patients diagnosed with pSS based on the 2016 pSS classification criteria. RESULTS:The study group consisted of 46 patients with early-onset pSS (≤35 years of age) and 32 patients with late-onset pSS (≥65 years of age). The study group was identified from a total of 228 patients diagnosed with pSS. There were no differences regarding the frequency of eye and mouth dryness, focus score (FS) ≥1 or anti-SSA/SSB antibodies depending on age. Rheumatoid factor (RF) was more common among older patients (p > 0.05). In the overall assessment of disease activity using European League Against Rheumatism (EULAR) Sjögren Syndrome Disease Activity Index (ESSDAI), no differences related to age were observed on the first and last visit (after 36 months on average). Lymphadenopathy and changes in the hematology domain (p < 0.05) were more common in patients with the early-onset phenotype. Changes in the lungs and musculoskeletal system occurred regardless of age. CONCLUSIONS:Patients with early-onset pSS differ from those with late-onset pSS in terms of higher incidence of peripheral lymphadenopathy and cytopenia. The involvement of lung tissue and joints as well as dryness symptoms are common in pSS regardless of age. The RF plays a role in the pathomechanism of pSS development.
10.17219/acem/140178
[Sjögren's syndrome with juvenile onset].
Kaleda M I,Nikishina I P,Latypova A N
Terapevticheskii arkhiv
Aim to analyze demographic data, clinical features and results of laboratory and instrumental examinations in children with primary and secondary Sjögren syndrome (SS). MATERIALS AND METHODS:The study included all consequently patients, who hospitalized to the pediatric department of V.A. Nasonova Scientific and Research Institute of Rheumatology from January 2013 to December 2018, which verified the diagnosis of the SS. RESULTS:The diagnosis of SS was established in 30 patients, among whom there were only 5 (16.7%) boys, the ratio of boys and girls was 1:5. According to the results of the examination, the following diagnoses were verified: 4 - primary SS, 9 - systemic lupus erythematosus with SS, 10 - juvenile rheumatoid arthritis with SS, 3 - mixed connective tissue disease, 3 - overlap syndrome, 1 - systemic sclerosis with SS. The median age of rheumatic disease onset was 10.4 (7.0; 13.75) years. The median of disease duration at the time of SS verification - 3.0 (0.85; 4.4) years. Recurrent parotitis were observed in 8 patients. 24 pts had isolated involvement of salivary glands, 6 - combined with lacrimal glands. Sicca syndrome was occurred in 8 patients. All patients had systemic manifestations: constitutional abnormalities - 50%, polyarthritis - 83.3%, lymphadenopathy - 73.3%, cutaneous involvement - 60%, pulmonary involvement - 23.3%. Of the hematological disorders, leuko/lymphopenia was more often recorded - in 30%, polyclonal hypergammaglobulinemia - in 26.7% of patients. ANA were detected in all cases, anti-Ro antibodies - 60%, a positive rheumatoid factor - 56.7% of patients. The most common combination of immunological disorders was the presence of ANA, RF and anti-Ro antibodies (40% of patients). The treatment for each patient was justified by the main manifestations and activity of rheumatic disease: 66.7% received nonsteroidal anti - inflammatory drugs, 80% - glucocorticoids, 46.7% - methotrexate, 20% - azathioprine, 43.3% - hydroxychloroquine, 10% - mycophenolatis mofetilum, 3.4% - cyclophosphamide. 66.7% of patients received the treatment of Biologics. CONCLUSIONS:Early diagnosis of SS in children with rheumatic diseases significantly affects to the choice of treatment and prognosis. In children the SS has no clinical manifestations for a long time. Such symptoms as a hypergammaglobulinemia, positive RF without persistant arthritis, nonspecific skin lesions, recurrent parotid swelling may help to diagnosis of SS.
10.26442/00403660.2019.05.000189
Primary Sjögren's Syndrome of Early and Late Onset: Distinct Clinical Phenotypes and Lymphoma Development.
Goules Andreas V,Argyropoulou Ourania D,Pezoulas Vasileios C,Chatzis Loukas,Critselis Elena,Gandolfo Saviana,Ferro Francesco,Binutti Marco,Donati Valentina,Zandonella Callegher Sara,Venetsanopoulou Aliki,Zampeli Evangelia,Mavrommati Maria,Voulgari Paraskevi V,Exarchos Themis,Mavragani Clio P,Baldini Chiara,Skopouli Fotini N,Fotiadis Dimitrios I,De Vita Salvatore,Moutsopoulos Haralampos M,Tzioufas Athanasios G
Frontiers in immunology
Objectives:To study the clinical, serological and histologic features of primary Sjögren's syndrome (pSS) patients with early (young ≤35 years) or late (old ≥65 years) onset and to explore the differential effect on lymphoma development. Methods:From a multicentre study population of 1997 consecutive pSS patients, those with early or late disease onset, were matched and compared with pSS control patients of middle age onset. Data driven analysis was applied to identify the independent variables associated with lymphoma in both age groups. Results:Young pSS patients (19%, n = 379) had higher frequency of salivary gland enlargement (SGE, lymphadenopathy, Raynaud's phenomenon, autoantibodies, C4 hypocomplementemia, hypergammaglobulinemia, leukopenia, and lymphoma (10.3% vs. 5.7%, p = 0.030, OR = 1.91, 95% CI: 1.11-3.27), while old pSS patients (15%, n = 293) had more frequently dry mouth, interstitial lung disease, and lymphoma (6.8% vs. 2.1%, p = 0.011, OR = 3.40, 95% CI: 1.34-8.17) compared to their middle-aged pSS controls, respectively. In young pSS patients, cryoglobulinemia, C4 hypocomplementemia, lymphadenopathy, and SGE were identified as independent lymphoma associated factors, as opposed to old pSS patients in whom SGE, C4 hypocomplementemia and male gender were the independent lymphoma associated factors. Early onset pSS patients displayed two incidence peaks of lymphoma within 3 years of onset and after 10 years, while in late onset pSS patients, lymphoma occurred within the first 6 years. Conclusion:Patients with early and late disease onset constitute a significant proportion of pSS population with distinct clinical phenotypes. They possess a higher prevalence of lymphoma, with different predisposing factors and lymphoma distribution across time.
10.3389/fimmu.2020.594096
Patients with early-onset primary Sjögren's syndrome have distinctive clinical manifestations and circulating lymphocyte profiles.
Wei Lin,Zhifei Xin,Xiaoran Ning,Meilu Liu,Yang Li,Yixuan Liu,Xiuying Ren,Yashuang Su,Jingjing Cao,Shaoying Guo,Liu Yang,Lijun Sun,Fengxiao Zhang,Wen Zhang
Rheumatology (Oxford, England)
OBJECTIVES:To further investigate the clinical characteristics and circulating lymphocyte profiles of patients with early-onset primary Sjögren's syndrome (pSS). METHOD:Data of 333 patients with pSS were analysed retrospectively. Early onset was defined as a pSS diagnosis at an age of 35 years or younger. The clinical, laboratory and immunophenotypic profiles of peripheral blood lymphocyte subsets were compared between early- and later-onset pSS. RESULTS:Thirty-six (10.81%) patients matched the definition of early-onset pSS, with age at disease onset being 28.97 (5.53) years. Elevated serum IgG level (77.14% vs 31.16%, P <0.001), low C3 (41.67% vs 20.20%, P =0.004) and C4 levels (27.78% vs 6.40%, P <0.001), anti-SSA positivity (91.67% vs 51.85%, P <0.001) and anti-SSB positivity (50% vs 20.54%, P <0.001) were more frequent in early-onset patients. The frequencies of hematological (80.56% vs 52.53%, P =0.001), renal (19.44% vs 5.05%, P =0.005) and mucocutaneous involvement (50% vs 22.56%, P <0.001) were significantly higher in the early-onset pSS group, which showed a higher 2010 EULAR SS Disease Activity Index (ESSDAI) [11(6.25-17) vs 7(3-12); P =0.003], compared with the later-onset group. In addition, profound CD4+ T-cell lymphopenia was found in patients with early-onset. CONCLUSIONS:Patients with early-onset pSS have distinctive clinical manifestations and greater activation of the cellular immune system, present with more severe clinical symptoms and immunological features, have increased activation of circulating T cells and have an unfavourable prognosis. Thus, they require more positive treatment with glucocorticoids and/or immunosuppressants and merit closer follow-up and regular monitoring.
10.1093/rheumatology/keab367
Early onset primary Sjögren syndrome, clinical and laboratory characteristics.
Yayla Müçteba Enes,Karaman Zehra,Torgutalp Murat,Keleşoğlu Dinçer Ayşe Bahar,Aydemir Gülöksüz Emine Gözde,Sezer Serdar,Şahin Eroğlu Didem,Yüksel Mehmet Levent,Turgay Tahsin Murat,Kınıklı Gülay,Ateş Aşkın
Clinical rheumatology
INTRODUCTION/OBJECTIVES:Primary Sjögren syndrome (pSS) is usually encountered between the fourth and sixth decades. It is known that the age of onset in autoimmune diseases may affect the clinical features. In this study, we aimed to investigate the clinical and laboratory characteristics of early onset pSS patients. METHOD:The data of 352 pSS patients were analyzed retrospectively. The patients were divided into two groups as those with the onset age of 35 or younger (early-onset) and those with the onset age of older than 35. The clinical, laboratory, and serological characteristics of the two groups were compared. p < 0.05 was considered statistically significant. RESULTS:Forty patients in the group with an onset age of 35 or younger (11.4%) and 312 patients with an onset age of older than 35 (88.6%) were analyzed. The frequency of skin (22.5% vs 1.9%, p < 0.001) and renal involvement (10% vs 2.2%, p = 0.026) was significantly higher in the early-onset group than the late-onset group. There was no significant difference between the two groups in terms of xerostomia, eye dryness, arthritis, and other systemic involvement. Anti-Ro52 positivity (p = 0.04), elevated serum IgG levels (p = 0.004), and low C4 (p = 0.002) presence were more frequent in the early-onset group. CONCLUSIONS:Consequently, it is seen that the clinical phenotype of early-onset pSS patients may be different to those with later onset. Especially the more frequent observation of poor prognostic factors at early-onset ages shows the necessity to monitor these patients more regularly. KEY POINTS:• The clinical and laboratory features of patients with early-onset primary Sjogren syndrome may differ from late-onset patients.
10.1007/s10067-020-05017-3