“…Valera et al, in Chile, in a retrospective review of 13 years, analyzing the medical records of 115 patients with PBC (110 women, aged between 30 and 76 years), found that 78% were symptomatic on presentation (itching, fatigue) and 56% of cases were AMA positive. Sjögren was present in 38%, hypothyroidism in 13%, scleroderma in 7%, and RA in 5% [41].…”
Section: Review Of the Literaturementioning
confidence: 99%
“…In some studies that analyzed the prevalence of autoimmune diseases in patients with PBC, pSS was the most prevalent. [18,41,46,47].…”
Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.
“…Valera et al, in Chile, in a retrospective review of 13 years, analyzing the medical records of 115 patients with PBC (110 women, aged between 30 and 76 years), found that 78% were symptomatic on presentation (itching, fatigue) and 56% of cases were AMA positive. Sjögren was present in 38%, hypothyroidism in 13%, scleroderma in 7%, and RA in 5% [41].…”
Section: Review Of the Literaturementioning
confidence: 99%
“…In some studies that analyzed the prevalence of autoimmune diseases in patients with PBC, pSS was the most prevalent. [18,41,46,47].…”
Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.
“…Patients should be followed closely and treated for consequences of chronic cholestasis, such as pruritus [16•], osteoporosis, and vitamin deficiencies. Steroid therapy may modestly improve liver biochemistries [3,4,[17][18][19][20][21], just as it does in AMA-positive PBC [22], but is best avoided because of the risk of accelerated bone density loss and other side effects associated with life-long steroid therapy. Portal hypertension, which may develop in advanced disease, is managed in the same manner as other liver diseases and will not be discussed here.…”
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