scholarly journals Hepatoportal Sclerosis in patient with Systemic Sclerosis and Sjögren’s Syndrome: a rare association

2021 ◽  
Vol 2 (3) ◽  
Author(s):  
Giorgio Soardo ◽  
◽  
Maria Orsaria ◽  
Laura Scatà ◽  
Debora Donnini ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Systemic Sclerosis ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Hepatoportal Sclerosis ◽  
Portal Veins ◽  
Portal Tracts ◽  
Sjögren‘S Syndrome

We present the case of a 51 years-old woman who was referred to our Liver Unit for suspected dysmetabolic liver disease. She had a previous diagnosis of Systemic Sclerosis and Sjögren’s Syndrome and had altered hepatic enzymes with a positive anti-Nuclear Antibodies centromeric pattern, anti-Cardiolipin Antibodies and anti b2 Glycoprotein I Antibodies. Despite complete liver assessment and disease staging was negative, the clinical course was complicated by the development of anemia, due to esophageal varices bleeding associated with worsening splenomegaly. Liver biopsy was key for reaching the diagnosis as it showed portal tracts with fibrous expansion, ductular proliferation and focal lympho-granulocytic infiltrate, reduced caliber of portal vessels, hypoplastic portal tracts, focal herniation, aberrant microvasculature and positive endothelial CD34 staining. Having ruled out any other cause of portal hypertension such as cirrhosis, blood diseases, occlusion of the hepatic and portal veins, etc., we finally concluded that the portal hypertension was due to hepatoportal sclerosis associated with Systemic Sclerosis and Sjögren’s Syndrome. Keywords: Idiopathic Non-Cirrhotic Portal Hypertension (INCPH; Hepatoportal Sclerosis (HPS); Systemic sclerosis (SSc); Sjögren’s Syndrome (SS).

Idiopathic portal hypertension associated with systemic sclerosis and Sjögren’s syndrome

2005 ◽  
Vol 24 (5) ◽  
pp. 544-547 ◽  
Author(s):  
Hiroyuki Kogawa ◽  
Kiyoshi Migita ◽  
Masahiro Ito ◽  
Yasushi Takii ◽  
Manabu Daikoku ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Systemic Sclerosis ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Idiopathic Portal Hypertension ◽  
Sjögren‘S Syndrome

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 Presence of microchimerism in labial salivary glands in systemic sclerosis but not in Sjögren's syndrome

2002 ◽  
Vol 46 (4) ◽  
pp. 1039-1043 ◽  
Author(s):  
Sélim Aractingi ◽  
Jean Sibilia ◽  
Véronique Meignin ◽  
David Launay ◽  
Eric Hachulla ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Salivary Glands ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

Sjögren's syndrome: An important confounder in evaluation of oral features in systemic sclerosis

Oral Diseases ◽  
2020 ◽  
Vol 26 (7) ◽  
pp. 1592-1593
Author(s):  
Katica Parat ◽  
Mislav Radić ◽  
Dijana Perković ◽  
Dolores Biočina Lukenda ◽  
Dusanka Martinović Kaliterna
Keyword(s):  
Systemic Sclerosis ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome
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