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Autoimmune hepatitis and systemic sclerosis: a new overlap syndrome?
I Marie1, H Levesque, J L Tranvouez
1Department of Internal Medicine, Centre Hospitalier Universitaire de Rouen-Boisguillaume, 76031 Rouen Cedex, France.
Rheumatology (Oxford, England)
|February 7, 2001
Summary
Autoimmune hepatitis can be a liver manifestation of systemic sclerosis (SSc). Early evaluation for SSc is recommended in patients with autoimmune hepatitis, as liver issues may appear before skin symptoms.
Area of Science:
- Rheumatology
- Hepatology
- Autoimmune Diseases
Background:
- Systemic sclerosis (SSc) is a connective tissue disease with diverse manifestations.
- The CREST variant of SSc involves calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.
- Liver involvement in SSc is uncommon but recognized.
Observation:
- Two patients with the CREST variant of SSc developed autoimmune hepatitis.
- In one case, liver disease preceded the characteristic skin manifestations of SSc.
Findings:
- Autoimmune hepatitis is a potential liver manifestation of SSc.
- Liver involvement in SSc may occur before skin symptoms are evident.
Implications:
- Consider SSc evaluation in patients with unexplained autoimmune hepatitis.
- Diagnostic workup for SSc should include clinical assessment, antinuclear antibody testing (particularly anticentromere antibodies), and nailfold capillaroscopy.
- Early suspicion of AIH in SSc patients with cytolytic hepatitis aids diagnosis and management.