Comorbidity of localized scleroderma and primary biliary cholangitis

Abstract

Background and objectives: Morphea is frequently associated with other autoimmune disorders. Little is known about the association of morphea and primary biliary cholangitis (PBC). The objective of this case series was to study the possible association of morphea with PBC and to identify risk factors.

Patients and methods: Patients with morphea were screened for anti-mitochondrial antibodies (AMA) by indirect immunofluorescence and/or immunoblot. Human leucocyte antigen (HLA) genotyping and deep sequencing for the HLA DRB1 subgroup were confirmed in AMA-positive patients.

Results: 6 of 91 patients (6.6 %) showed positivity for AMA, and 4 (4.39 %) had PBC. The mean age of AMA-positive patients was 69.0 years. Of 6 AMA-positive patients, common predisposing alleles (HLA DRB1*15: 01 and HLA DRB1*08) were detectable in two patients. One patient had predisposing alleles for both diseases (HLA DRB1*03: 01 and HLA DRB1*14). One patient had a PBC-conferring allele. Female gender, menopause and tendency for remission of morphea were common in all patients.

Conclusion: The coexistence of morphea, AMA positivity and PBC is a rare but possible association. Common predisposing HLA alleles might interact in such a simultaneous manifestation. We suggest AMA screening for female patients with generalized morphea before the initiation of methotrexate.