Features of Sjögren's syndrome in patients with primary biliary cirrhosis

Uddenfeldt, P; Danielsson, Åke; Forssell, Åke; Holm, Margo B.; Östberg, Yngve

doi:10.1111/j.1365-2796.1991.tb00470.x

Cited by 52 publications

(28 citation statements)

References 20 publications

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“…Among autoimmune conditions found in PBC cases, Raynaud syndrome (12%) and Sjögren syndrome (10%) were the most frequent, in accordance with previous reports. 22,23 We note that other studies reported higher prevalence rates but were based on a smaller number of probably select cases. We found that 27 cases (3%) with PBC reported a diagnosis of SLE.…”

Section: Discussionmentioning

confidence: 88%

Risk Factors and Comorbidities in Primary Biliary Cirrhosis: A Controlled Interview-Based Study of 1032 Patients *

et al. 2005

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Section: Discussionmentioning

confidence: 88%

Risk Factors and Comorbidities in Primary Biliary Cirrhosis: A Controlled Interview-Based Study of 1032 Patients *

et al. 2005

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“…In contrast to AIH, the most frequently associated CAIDs in PBC is the Sjogren's syndrome, which has been reported in the literature with a wide proportion of cases ranging from 20% to nearly 80% [91][92][93][94][95][96] . Two large cohort studies from the United Kingdom and Italian groups have identified Sjogren's syndrome as the most prevalent CAIDs with 25 and 34% in patients with PBC respectively [97,98] .…”

Section: Comparisons With Pbcmentioning

confidence: 99%

“…Two large cohort studies from the United Kingdom and Italian groups have identified Sjogren's syndrome as the most prevalent CAIDs with 25 and 34% in patients with PBC respectively [97,98] . The target antigen in salivary gland ducts of patients with Sjogren's has been shown in the bile duct epithelium of PBC patients [96] . In a population-based cohort study in the United Kingdom, 53% of patients with PBC had one or more CAIDs [97] .…”

Section: Comparisons With Pbcmentioning

confidence: 99%

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Wong

Heneghan²

2015

Dig Dis

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For many patients with autoimmune hepatitis (AIH), the presence of extrahepatic features is well recognised both at the time of presentation and during long-term follow-up. Concomitant ‘autoimmune disorders' have been described in 20-50% of patients with AIH, both in adults and children. Indeed, the presence of these associated phenomena has been incorporated into both the original and revised International AIH group scoring systems as an aid to codifying the diagnosis. In acute index presentations, non-specific joint pains sometimes flitting in nature have been reported in 10-60% of patients, and while joint swelling is uncommon, rheumatoid arthritis and mixed connective tissue disease have been reported in 2-4% of patients with AIH. For a majority of patients, these joint symptoms resolve within days of the introduction of immunosuppressive therapy. Rarer features at index presentation include a maculopapular skin rash and unexplained fever, which are features that tend to resolve quickly with treatment. Interestingly, joint pain and stiffness are also well recognised in the context of steroid withdrawal and cessation in AIH. The occasional co-presentation of AIH with coeliac disease is clinically important (1-6%), since for some patients, there is a risk of immunosuppression malabsorption, thus delaying effective treatment. Similarly, the co-existence of selective IgA deficiency (IgAD) can occur in patients with coeliac disease or in isolation. Selective IgAD as a co-existing extraheaptic feature seems to be more common in paediatric patients with AIH. For these patients, they are at an increased risk of respiratory and sinus infections. Although, typically associated with primary sclerosing cholangitis, the presence of inflammatory bowel disease (IBD; both Crohn's disease and ulcerative colitis) has been described in 2-8% of patients with AIH. Interestingly, for patients with autoimmune sclerosing cholangitis, a distinct pattern of IBD has been recently described. Other conditions have been reported at a lower frequency, including Sjogren's syndrome 1-7%, systemic lupus erythematosus 1-3% and glomerulonephritis 1%. Rarer still and at a frequency of <1% include fibrosing alveolitis, haemolytic anaemia, uveitis, mononeuritis multiplex, polymyositis and multiple sclerosis. In contrast, the reported associations between AIH and thyroiditis 8-23%, diabetes 1-10% and psoriasis 3% are commonly seen and notable in clinical practice.

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“…Various anti thyroid antibodies, such as antimicrosomal or antithyroglobulin antibodies, are present in a large percentage of patients [ 17,47], Associations of scleroderma, arthropathy and Sjogren's syndrome with PBC have been well described. Scleroderma may be present in 3-4% of PBC patients [48] while symptom atic inflammatory arthritis is reported in 4 -42% of patients [49], Sjorgren's syndrome de fined by the presence of (1) dry eyes (kerato conjunctivitis sicca) and/or xerostomia, and (2) autoimmune disease is seen in 72-100% of patients [1,50].…”

Section: Associated Diseasesmentioning

confidence: 99%

Primary Biliary Cirrhosis

Laurin

Lindor

1994

Dig Dis

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Primary biliary cirrhosis is a chronic, usually progressive, cholestatic liver disease of presumed autoimmune etiology that affects predominantly young and middle-aged women. It is nearly always associated with an antibody directed against a component of the pyruvate dehydrogenase complex located on the inner wall of the mitochondria. The disease is associated with a number of other associated autoimmune disorders. No totally effective medical treatment has been established for the disease, although ursodeoxycholic acid appears promising. Complications of cholestasis such as fat malabsorption and fat-soluble vitamin deficiency should be excluded or corrected when found. Individual patient prognosis varies. Several models for estimating individual patient survival are available. Liver transplantation is recognized as a procedure to extend and improve the quality of life for patients with advanced disease.

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Features of Sjögren's syndrome in patients with primary biliary cirrhosis

Cited by 52 publications

References 20 publications

Risk Factors and Comorbidities in Primary Biliary Cirrhosis: A Controlled Interview-Based Study of 1032 Patients *

Risk Factors and Comorbidities in Primary Biliary Cirrhosis: A Controlled Interview-Based Study of 1032 Patients *

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Primary Biliary Cirrhosis

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