Giant Cell Hepatitis - A Rare Association with Connective Tissue Disease

A 68-year-old gentleman presented to hepatology department with asymptomatic year-long history of stably deranged liver function tests. His peak alkaline phosphatase (ALP), was 828 with alanine transaminase (ALT) of 141. Full liver workup was negative; hence, a liver biopsy was organised, which conf...

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Bibliographic Details
Published in:Mediterranean journal of rheumatology 2019-12, Vol.30 (4), p.224-227
Main Authors: Rauf, Maria, Sen, Sambit, Levene, Adam, Nisar, Muhammad K
Format: Article
Language:English
Subjects:
Adults
Antibodies
Anticoagulants
Autoimmune diseases
Case Report
Conflicts of interest
Connective tissue diseases
Cytomegalovirus
Hepatitis
Hepatology
Hospitals
Hypertension
Inflammation
Liver diseases
Lung diseases
Lupus
Rheumatic diseases
Rheumatology
Viral infections
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Summary:A 68-year-old gentleman presented to hepatology department with asymptomatic year-long history of stably deranged liver function tests. His peak alkaline phosphatase (ALP), was 828 with alanine transaminase (ALT) of 141. Full liver workup was negative; hence, a liver biopsy was organised, which confirmed giant cell hepatitis (GCH). A computed tomography (CT) scan revealed non-specific interstitial pneumonitis (NSIP) pattern interstitial lung disease supported by lung function tests. Antibody testing showed strongly positive antinuclear antibody (ANA) with anti-polymyositis/scleroderma (anti-PM-SCL) antibody. Clinical picture was in keeping with likely undifferentiated connective tissue disease (UCTD) with polyarthralgia, early morning stiffness, Raynaud's and nailfold infarcts with capillaritis on nail bed examination. Further testing confirmed triple-positive antiphospholipid antibodies twice 12 weeks apart (immunoglobulin M [IgM] anti beta-2 glycoprotein antibodies, lupus anticoagulant and IgM anticardiolipin antibody). He was treated with mycophenolate and hydroxychloroquine with resolution of symptoms. Giant cell hepatitis is uncommon, with only 100 cases reported worldwide. To our knowledge, this is the only report of GCH in the context of UCTD, highlighting the significance of careful evaluation of liver disease overlap and the successful role of mycophenolate mofetil (MMF) in this setting.
ISSN:2529-198X
2529-198X
DOI:10.31138/MJR.30.4.224