Autoimmune hepatitis in primary Sjögren's syndrome: Pathological study of the livers and labial salivary glands in 17 patients with primary Sjögren's syndrome

Matsumoto, Takeshi; Morizane, Toshio; Aoki, Yuuji; Yamasaki, Shigetaka; Nakajima, Mari; Enomoto, Nobuyuki; Kobayashi, Shigeto; Hashimoto, Hiroshi

doi:10.1111/j.1440-1827.2005.01790.x

Cited by 54 publications

(31 citation statements)

References 16 publications

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“…Liver involvement is considered to be the most common non-exocrine feature in pSS, presenting as abnormal liver function tests in 49% of cases [37]. Both AIH and PBC are major liver diseases in patients with pSS.…”

Section: Discussionmentioning

confidence: 99%

The Development of Autoimmune Hepatitis and Primary Biliary Cirrhosis Overlap Syndrome During the Course of Connective Tissue Diseases: Report of Three Cases and Review of the Literature

et al. 2009

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Section: Discussionmentioning

confidence: 99%

The Development of Autoimmune Hepatitis and Primary Biliary Cirrhosis Overlap Syndrome During the Course of Connective Tissue Diseases: Report of Three Cases and Review of the Literature

et al. 2009

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“…In a recent nationwide study in Japan involving 1,056 patients with AIH, Sjogren's was the second commonest CAIDs reported with the frequency of 7.2% after autoimmune thyroiditis [14] . In a study of 17 Sjogren's patients with elevated liver enzymes, AIH was diagnosed in 9 of these patients and the histological examination of liver and labial salivary glands in these patients revealed similar inflammatory reactions in both organs, including infiltration of both lymphocytes and plasma cells, with a predominance of CD3+ T cells, indicating the close relationship between AIH and Sjogren's [66] .…”

Section: Connective Tissue Disorders (Ctds)mentioning

confidence: 96%

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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“…In this case, it was difficult to differentiate AIH and reactive or non-specified hepatitis associated with RA or SjS initially, because patients with RA or SjS frequently show positive ANA and an increased level of IgG, which are important in the diagnosis of AIH. Therefore, histology obtained from liver biopsy was important in the present case (13)(14)(15). The histology of hepatic lesions due to SjS or RA has been reported as non-specific inflammation with infiltration of mononuclear cells and with fatty change (13,16,17).…”

Section: Discussionmentioning

confidence: 93%

Improvement of Rheumatoid Arthritis and Autoimmune Hepatitis in a Patient Treated with the Tumor Necrosis Factor Inhibitor, Etanercept

et al. 2011

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We report a case of rheumatoid arthritis (RA) with autoimmune hepatitis (AIH) and Sjogren syndrome (SjS) that was treated with the tumor necrosis factor (TNF) inhibitor, etanercept (ETN). Both RA activity and transaminase levels improved as a result of treatment. Follow-up liver biopsy showed improvement of hepatitis. Although the efficacy of anti-TNF for RA patients with AIH remains controversial, this case suggests that treatment with ETN may result in a favorable clinical course in a certain subset of patients with RA and AIH.

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Autoimmune hepatitis in primary Sjögren's syndrome: Pathological study of the livers and labial salivary glands in 17 patients with primary Sjögren's syndrome

Cited by 54 publications

References 16 publications

The Development of Autoimmune Hepatitis and Primary Biliary Cirrhosis Overlap Syndrome During the Course of Connective Tissue Diseases: Report of Three Cases and Review of the Literature

The Development of Autoimmune Hepatitis and Primary Biliary Cirrhosis Overlap Syndrome During the Course of Connective Tissue Diseases: Report of Three Cases and Review of the Literature

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Improvement of Rheumatoid Arthritis and Autoimmune Hepatitis in a Patient Treated with the Tumor Necrosis Factor Inhibitor, Etanercept

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