scholarly journals Autoimmune rheumatic diseases and Klinefelter syndrome Autoimunitné reumatické choroby a Klinefelterov syndróm

2016 ◽  
Vol 63 (2) ◽  
pp. 18-22 ◽  
Author(s):  
I. Lazúrová ◽  
J. Rovenský ◽  
R. Imrich ◽  
S. Blažíčková ◽  
Z. Lazúrová ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Rheumatic Diseases ◽  
Lupus Erythematosus ◽  
Sjögren's Syndrome ◽  
Klinefelter Syndrome ◽  
Connective Tissue Diseases ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Autoimmune Rheumatic Diseases ◽  
Sjögren‘S Syndrome

AbstractThe article summarizes data on the association of Klinefelter syndrome (KS) with autoimmune rheumatic diseases, that is rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), polymyositis/dermatomyositis, systemic sclerosis (SSc), mixed connective tissue diseases (MCTD), Sjogren’s syndrome and antiphospholipid syndrome (APS). Recently, a higher risk for RA, SLE and Sjogren’s syndrome in patients with KS has been clearly demonstrated. However, the association of other autoimmune rheumatic disorders such as dermatomyositis/polymyositis, SSc, MCTD and APS is reported only casually. Based on the hormonal changes in KS, there are suggestions that low androgen and higher estrogen levels might be a predisposing factor for the development of autoimmune diseases, but evidence for the association is poor. Epidemiologic studies on larger cohorts of patients are required.

scholarly journals AB0609 THE SPECTRUM OF ANTINUCLEAR ANTIBODIES IN PATIENTS WITH SYSTEMIC SCLEROSIS POSITIVE FOR ANTI-U1RNP

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1600.1-1601
Author(s):  
R. Shayakhmetova ◽  
L. P. Ananyeva ◽  
O. Koneva ◽  
M. Starovoytova ◽  
O. Ovsyannikova ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Sjögren’S Syndrome ◽  
Lupus Erythematosus ◽  
Clinical Picture ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Normal Limits ◽  
Musculoskeletal Manifestations ◽  
Sjögren‘S Syndrome

Background:Patients with systemic sclerosis positive for anti-U1RNP have special clinical picture and disease progression. The autoimmune profile in this group is poorly understood.Objectives:The purpose of our work was to study the level of major autoantibodies in patients with systemic sclerosis positive for anti-U1RNP.Methods:The study included 80 patients (71 women and 9 men, mean age 44,5±14 years) positive for antibodies to RNP and meeting the criteria of the systemic sclerosis (ACR/EULAR 2013). Patients were examined for autoantibodies: RF, ACCP, ACA, anti-Scl70, anti-RNAP-III, anti-Ro, anti-La, anti-dsDNA, anti-Sm, ACL, anti-Jo1. 44 patients were examined in dynamics in 24 months.Results:In the study group the clinical picture was dominated by inflammatory musculoskeletal lesions (synovitis and myopathy), skin manifestations were poorly expressed. Interstitial lung disease was detected in 68% of cases. Overlaps (34%) with other rheumatic diseases (rheumatoid arthritis, systemic lupus erythematosus) and combination with Sjogren’s syndrome (32.5%) were frequently noted. Other antibodies were often detected: commonly - RF (31%), anti-Ro (38%), anti-dsDNA (42%), rarely - anti-Sm (11%), ACCP (8%), anti-La (8%), ACA (6%), anti-Scl70 (6%), AKL (2%). Anti-Jo1 and anti-RNAP-III were not detected at all. In patients with systemic sclerosis highly-positive for anti-U1RNP (more than 2 upper normal limits) RF, anti-Ro, anti-dsDNA were significantly more common in comparison with low-positive(p=0.00). In dynamics 80% of patients maintained anti-U1RNP, while other autoantibodies were detected with the same frequency. In patients with initially low titer of anti-U1RNP, their disappearance was noted.Conclusion:Patients with systemic sclerosis positive for anti-U1RNP differ in the predominance of inflammatory musculoskeletal manifestations and frequent combination with Sjogren’s syndrome and overlaps. Highly positivity for anti-U1RNP is accompanied by a persistent increase in RF, anti-Ro, anti-dsDNADisclosure 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.

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