scholarly journals The Involvement of Innate and Adaptive Immunity in the Initiation and Perpetuation of Sjögren’s Syndrome

2021 ◽  
Vol 22 (2) ◽  
pp. 658
Author(s):  
Clara Chivasso ◽  
Julie Sarrand ◽  
Jason Perret ◽  
Christine Delporte ◽  
Muhammad Shahnawaz Soyfoo
Keyword(s):  
Immune System ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Adaptive Immune System ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sicca Syndrome ◽  
Neurological Involvement ◽  
Adaptive Immune ◽  
Sjögren‘S Syndrome

: Sjogren’s syndrome (SS) is a chronic autoimmune disease characterized by the infiltration of exocrine glands including salivary and lachrymal glands responsible for the classical dry eyes and mouth symptoms (sicca syndrome). The spectrum of disease manifestations stretches beyond the classical sicca syndrome with systemic manifestations including arthritis, interstitial lung involvement, and neurological involvement. The pathophysiology underlying SS is not well deciphered, but several converging lines of evidence have supported the conjuncture of different factors interplaying together to foster the initiation and perpetuation of the disease. The innate and adaptive immune system play a cardinal role in this process. In this review, we discuss the inherent parts played by both the innate and adaptive immune system in the pathogenesis of SS.

scholarly journals AB0328 CLINICAL AND SEROLOGICAL DIFFERENCES BETWEEN PRIMARY SJOGREN’S SYNDROME PHENOTYPES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1189.1-1189
Author(s):  
X. E. Larco Rojas ◽  
E. Diez Alvarez ◽  
C. Moriano ◽  
A. López Robles ◽  
T. Pérez Sandoval
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Age Groups ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Sjögren’S Syndrome ◽  
The Other ◽  
Neurological Involvement ◽  
Primary Sjogren’S Syndrome ◽  
Sjögren‘S Syndrome

Background:Primary Sjogren’s Syndrome (PSS) is an autoimmune and lymphoproliferative disease with a heterogeneous presentation. It has been postulated that there may be different phenotypes, in some cases presenting a more aggressive disease with systemic manifestations and a higher risk of developing complications. This phenotype has been associated with a higher autoimmune load and an earlier age of presentation. Furthermore, the presence of anti La + has been related to an increased risk of developing Lymphoma.Objectives:To describe the phenotypic characteristics of seronegative PSS in a sample of patients from our practice. To compare the clinical and laboratory characteristics between patients with Ro + / La + and Ro + / La- antibodies. To Analyze if there are differences in patients diagnosed at an early age, compared to a later age.Methods:Clinical and serological characteristics of patients with the diagnosis of PSS were collected from the Rheumatology database of León`s Hospital between 2014-2020. All patients who met the ACR / EULAR 2016 criteria were classified as seronegative Sjogren.In the group of patients with positive autoimmunity, anti-Ro + / La + were compared with the anti-Ro + / La- patients and by age, stratifying them into the following groups: 0-49; 50-69 and> 70 years. The clinical variables analyzed were: glandular inflammation, Raynaud’s phenomenon (RP), pulmonary and neurological involvement, presence of Lymphoma and other tumours. The serological variables were: positivity of ANA, Rheumatoid Factor (RF), hypocomplementemia, hypergammaglobulinemia and B2 microglobulin.Results:72 patients were analysed, 9 were excluded because didn’t meet the criteria. Of the remaining: 90,4% were women, with a mean age of 58,7+/-15,8 years, 12,6% (8) were seronegative. In the seronegative group 25% presented lung involvement (Lymphoid Interstitial Pneumonia), 50% presented with glandular inflammation and only one patient had RP. As complications 1 patient presented Lymphoma and 1 Breast Carcinoma.58,7% (37) Ro + / La + and 28,5% (18) Ro + / La- patients were identified, no statistically significant differences were found between the two groups when comparing: glandular inflammation (8/37 vs 2/18, p = >0.05) RP (9/37 vs 4/18, p = >0.05), pulmonary involvement (5/37 vs. 6/18, p = >0.05), neurological involvement (2/37 vs. 1/18, p = >0.05), presence of Lymphoma (2/37 vs. 0 / 18, p = >0,05), other tumours (2/37 vs 3/18, p = >0.05), ANA positivity (36/37 vs 16/18, p =>0,05), Hypocomplementemia (4/37 vs 3/18, p =>0.05) and Hypergammaglobulinemia (20/37 vs 10/18, p =>0.05). But a higher frequency of positive RF linked to anti La positivity (29/37 vs 6 / 18p = 0.002) was found.When comparing by age groups, the association between RF + and La + remained in the group of 50-69 years (15/18 vs 3/18, p = 0.002) while in the other age groups there were no statistically significant differences. We also observed an increasing trend of the levels of B2microglobulin in La+ patients and later age (p=0,04)Conclusion:The presence of anti La + seems to be associated with other components of autoimmunity such as RF in patients with PSS, although this study did not show a relation with a higher frequency of complications or systemic disease. Also, the presence of La+ at older ages was associated with higher levels of B2 microglobulin. We didn’t find differences with the other described markers of B cell reactivation. Findings differ from those found in the literature, which may be largely due to sample size.References:[1]Quartuccio L., Baldini C., Bartoloni E., et al. Anti-SSA/SSB-negative Sjogren’s syndrome shows a lower prevalence of lymphoproliferative manifestations, and a lower risk of lymphoma evolution. Autoimmunity Reviews 14 (2015) 1019–1022.[2]Quartuccio L, Isola M, Baldini C, Priori R, Bartoloni Bocci E, Carubbi F, et al. Biomarkers of lymphoma in Sjögren’s syndrome and evaluation of the lymphoma risk in prelymphomatous conditions: results of a multicenter study. J Autoimmun 2014; 51:75–80.Disclosure of Interests:None declared

scholarly journals Doença Intersticial Pulmonar Grave e Mania Induzida por Corticosteróides em Doente com Lúpus Eritematoso Sistémico e Síndrome de Sjögren Secundária

2017 ◽  
Vol 30 (3) ◽  
pp. 246 ◽  
Author(s):  
Sofia Silvério Serra ◽  
Teresa Pedrosa ◽  
Sandra Falcão ◽  
Jaime Cunha Branco
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Sjögren’S Syndrome ◽  
Lupus Erythematosus ◽  
Sjögren's Syndrome ◽  
Manic Episode ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Neurological Involvement ◽  
Systemic Lupus ◽  
Sjögren‘S Syndrome

Interstitial lung disease occurs in up to 25% of patients with Sjögren’s syndrome and 2% - 8 % of patients with systemic lupus erythematosus. Corticosteroid therapy remains the main treatment for systemic lupus erythematosus. However, it can be associated with several neuropsychiatric disorders especially with prednisolone at a dose of more than 40 mg/day. We present the case of a 51-year-old patient with systemic lupus erythematosus and secondary Sjögren’s syndrome with severe pulmonary involvement four years after the diagnosis. Chest computed tomography revealed neofibrosis and ground glass appearance pattern. After increasing the dose of prednisolone to 60 mg/day, the patient presented a manic episode. There was need of hospitalization and the situation was considered to be secondary to corticosteroids at high doses. Central neurological involvement by organic disease was excluded.We introduced monthly perfusion of cyclophosphamide for six months and later started mycophenolate mofetil 2 g/day, reducing prednisolone to 10 mg/day and maintaining hydroxychloroquine 400 mg/day, with control of disease activity.

scholarly journals Discrimination between Sjögren's and Non-Sjögren's Sicca Syndrome by Sialoscintigraphy and Antibodies against α-Fodrin and Ro/La Autoantigens

2009 ◽  
Vol 37 (4) ◽  
pp. 1088-1096 ◽  
Author(s):  
K-S Chen ◽  
M-C Jiang ◽  
C-J Li ◽  
O-K Liu ◽  
C-SS Tsai
Keyword(s):  
Salivary Gland ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sicca Syndrome ◽  
Dry Eyes ◽  
Sicca Symptoms ◽  
Grade Iii ◽  
Sjögren‘S Syndrome

Both Sjögren's syndrome (SS) and non-Sjögren's syndrome (NSS) can present with the sicca symptoms of dry eyes and a dry mouth but they are distinct pathological entities that require diagnostic discrimination. This study included 82 sicca syndrome patients and examined the ability of sialoscintigraphy and antibodies against the autoantigens α-fodrin, Ro and La to discriminate between SS and NSS. A total of 30.8% of SS patients compared with 58.8% of NSS patients were α-fodrin positive. The prevalence of Ro positivity was 69.4% for SS patients compared with 0% for NSS patients. The prevalence of La positivity was 52.4% for SS compared with 0% for NSS patients. Sialoscintigraphy showed that more NSS patients had grade III salivary gland impairment compared with SS patients (64.7% versus 19.4%). These data suggest that using sialoscintigraphy in combination with measuring the levels of serum α-fodrin, Ro and La might be useful for SS and NSS discrimination.

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