scholarly journals A rare case of systemic sclerosis complicated with multiple autoimmune diseases (Sjögren's syndrome, Graves' disease, and primary biliary cirrhosis)

2004 ◽  
Vol 14 (6) ◽  
pp. 476-479 ◽  
Author(s):  
Noriko Iikuni ◽  
Midori Hanzawa ◽  
Shuji Ohta ◽  
Makoto Nishinarita
Keyword(s):  
Systemic Sclerosis ◽  
Primary Biliary Cirrhosis ◽  
Autoimmune Diseases ◽  
Rare Case ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Graves Disease ◽  
Biliary Cirrhosis ◽  
Sjögren‘S Syndrome

scholarly journals AB0447 PREVALENCE OF PRIMARY BILIARY CIRRHOSIS IN SYSTEMIC SCLEROSIS AND SJÖGREN’S SYNDROME OVER TIME: A SYSTEMATIC REVIEW

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1251.1-1251
Author(s):  
S. Upadhyaya ◽  
D. Starcevic ◽  
M. Turk ◽  
J. Pope
Keyword(s):  
Systematic Review ◽  
Systemic Sclerosis ◽  
Primary Biliary Cirrhosis ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Biliary Cirrhosis ◽  
Prevalence Rates ◽  
Over Time ◽  
Sjögren‘S Syndrome

Background:Primary biliary cirrhosis (PBC) is a rare slowly progressive autoimmune disease characterized by inflammatory destruction and fibrosis of intrahepatic bile ducts. It is known to coexist together with rheumatological conditions such as Sjögren’s syndrome (SS) and systemic sclerosis (SSc). There is a wide range in reported prevalence of disease overlap with these entities; however, the exact prevalence rates remain unclear.Objectives:The objectives were to determine the prevalence of: 1) PBC in patients with SS and SSc (and the subsets of limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc)), 2) SSc and SS in patients with PBC, and 3) to analyze changes in frequency over time. SSc occurs in 3/10,000 and PBC in 4-40/10,000 but these rare autoimmune diseases are known to coexist together. We speculated that there could be more cases diagnosed due to increasing availability of standardized antibody tests such as ANA, centromere antibodies, ENA and mitochondrial antibodies.Methods:A systematic review of the literature was performed using Medline, EMBASE, CINAHL, and the Cochrane Library databases up till June 16, 2020. Only full text articles in the English language with at least 40 patients were included. Cohorts, case series, cross-sectional studies, correspondences and registries with reported prevalence rates of both PBC in patients with SS and SSc as well as SSc and SS in patients with PBC were included. Data on frequency of co-existent diseases was studied by year of publication to determine if prevalence changed over time using linear regression. We used the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) checklist to assess the quality of the studies.Results:Of 2876 citations identified, 67 were included in the analysis (n=33 for PBC, 15 for SSc, 18 for SS and 1 for SSc/SS). STROBE checklist scores ranged from 7-21. The prevalence of PBC was 5% in patients with SSc. Within the subsets, the prevalence of PBC in lcSSc was 8% and in dcSSc was 1%. In patients with SS, the prevalence of PBC was 4%. The prevalence of SSc overall in those with PBC was 5% and, within the subsets was 6% in lcSSc and 0% in dcSSc. The prevalence of SS in PBC was 18%. There was also no significant association between year of publication and prevalence. There was a lack of standardized definitions so misclassification may have occurred.Conclusion:PBC is increased in SSc but mostly in the lcSSc subset. SS in PBC is common at nearly 1 in 5. Over the years, there was no change in the prevalence of PBC in SSc indicating stability over time.Acknowledgements:Meagan Stanley, Western University Librarian.Disclosure of Interests:None declared.

Associated Autoimmune Diseases in Systemic Sclerosis Define a Subset of Patients with Milder Disease: Results from 2 Large Cohorts of European Caucasian Patients

2010 ◽  
Vol 37 (3) ◽  
pp. 608-614 ◽  
Author(s):  
JÉRÔME AVOUAC ◽  
PAOLO AIRÒ ◽  
PHILIPPE DIEUDE ◽  
PAOLA CARAMASCHI ◽  
KIET TIEV ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Digital Ulcers ◽  
Biliary Cirrhosis ◽  
Caucasian Patients ◽  
Sjögren‘S Syndrome

Objective.To assess the prevalence and potential associations with the systemic sclerosis (SSc) phenotype of additional autoimmune diseases (AID).Methods.A multicenter study was performed in France and Italy to recruit consecutive European Caucasian patients with SSc systematically assessed for the coexistence of predefined AID known to occur with connective tissue diseases.Results.We recruited 585 French and 547 Italian patients with SSc. Specific AID were found in 114/585 (19%) French and 179/547 (33%) Italians with SSc (p < 0.0001). Sjögren’s syndrome and thyroiditis were the predominant AID in both cohorts (12% for Sjögren’s syndrome and 6% for thyroiditis in the combined populations). The frequency of myositis, primary biliary cirrhosis, rheumatoid arthritis, and systemic lupus erythematosus was low (< 4%) and similar in both cohorts. The coexistence of at least 1 of the AID in the whole cohort was associated in multivariate analysis with the limited cutaneous subtype, the presence of antinuclear antibodies, and a lower prevalence of digital ulcers.Conclusion.Our study shows that 21% of this large series of European Caucasian patients with SSc have developed at least 1 AID. This latter condition identified a subset of patients with milder disease. Thus, associations of AID and autoimmune background in SSc have to be considered for further therapeutic and biological investigations in SSc.

Primary biliary cirrhosis and Sjogren's syndrome

1989 ◽  
Vol 2 (4) ◽  
pp. 611
Author(s):  
J Hamburger ◽  
A.J.C. Potts ◽  
J.B. Matthews ◽  
D.G.I. Scott ◽  
C. Vickers ◽  
...  
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Biliary Cirrhosis ◽  
Sjögren‘S Syndrome
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