A 28-year-old woman with a combined hepatitic/cholestatic syndrome

Wurbs, D

doi:10.1016/0270-9139(95)90170-1

Cited by 8 publications

(12 citation statements)

References 2 publications

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“…Sporadic case reports and small series have highlighted the presence of a condition in adults with features of both AIH and PSC. [65][66][67][68][69][70][71] Roughly half of the reported cases suffered from inflammatory bowel disease, and there is an overall male predominance in keeping with PSC. Of 16 cases described in detail, 10 were diagnosed initially as AIH, with subsequent development of diagnostic features of PSC.…”

Section: Psc/aih Overlapsmentioning

confidence: 99%

Autoimmune overlap syndromes

2001

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Section: Psc/aih Overlapsmentioning

confidence: 99%

Autoimmune overlap syndromes

2001

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“…Autoimmune hepatitis may be overlapped by other liver diseases with proven or possible autoimmune background, such as primary biliary cirrhosis or primary sclerosing cholangitis. 6–9 …”

Section: Introductionmentioning

confidence: 99%

Wilson’s disease with superimposed autoimmune features: Report of two cases and review

Milkiewicz

Saksena

Hübscher

et al. 2000

J of Gastro and Hepatol

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We describe two females, 15 and 23 years old, respectively, who presented with classical features of Wilson's disease (WD) and several features of autoimmune hepatitis (AIH). The first patient was initially diagnosed as AIH and treated with prednisolone which caused clinical improvement, with an increase of serum albumin from 22 to 30 g/L, and a decrease of aspartate aminotransferase from 103 to 47 U/L. Subsequent diagnosis of WD and introduction of penicillamine gave excellent improvement and complete normalization of liver function tests. The second patient, at first also diagnosed as having AIH, was treated with steroids and azathioprine with initial improvement, but subsequent deterioration. The diagnosis of WD was made 2 years after initial diagnosis of AIH, as the patient reached end-stage liver disease and required a transplant. Therefore, d-penicillamine treatment was not attempted. We conclude that, in patients with AIH, a thorough screening for WD is necessary, particularly when the response to steroid therapy is poor. Conversely, in patients suffering from WD with superimposed features of AIH, a combination of steroids and penicillamine may be of benefit.

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“…In our case, however, the unexpected occurrence of PSC was suggested not only by the development of cholestasis [12], but also by ANCA positivity, a serological conversion that has never been described before in literature.…”

Section: Discussionmentioning

confidence: 57%

“…The strong association of PSC with a series of autoimmune diseases underlines the role of immunological alterations in the pathophysiology of the disease [12]. Moreover, specific autoantibodies can be found in patients with PSC, i.e., p-ANCA, anticolon antibodies and anti-neutrophil nuclear antibodies with a high frequency as well as anti-mitochondrial auto-antibodies, ANAs and SMAs with a lower frequency .…”

Section: Discussionmentioning

confidence: 98%