Hepatitis, cholangitis, pulmonary hypertension, digital gangrene, and conjunctivitis sicca in a woman with anticentromere antibodies

Chang-Youh Tsai; An-Ping Huo; Chung-Tei Chou; Tsai-Hung Wu; Ying-Yang Tsai

doi:10.1016/j.ejim.2006.02.003

Hepatitis, cholangitis, pulmonary hypertension, digital gangrene, and conjunctivitis sicca in a woman with anticentromere antibodies

Eur J Intern Med. 2006 Aug;17(5):360-2. doi: 10.1016/j.ejim.2006.02.003.

Authors

Chang-Youh Tsai¹, An-Ping Huo, Chung-Tei Chou, Tsai-Hung Wu, Ying-Yang Tsai

Affiliation

¹ Division of Allergy, Immunology, and Rheumatology, Department of Medicine, Taipei Veterans General Hospital, 201, Shih-Pai Road, Section 2, Taipei 112, Taiwan. cytsai@vghtpe.gov.tw

PMID: 16864013
DOI: 10.1016/j.ejim.2006.02.003

Abstract

A 46-year-old woman presented with chronic fluctuated liver function impairment, Raynaud's phenomenon, digital gangrene, pulmonary hypertension, and intense pruritus within a period of 2 years. Laboratory investigations revealed antinuclear antibodies, anticentromere antibodies (ACA), hypergammaglobulinemia, lymphocytic infiltration of the liver parenchyma, and mild cholangitis. The associated symptoms included thyroiditis, conjunctivitis sicca, xerostomia, and polyarthralgia. There was no conspicuous sclerodactyly, calcinosis, or dysphagia. The symptoms were relieved with intravenous, as well as oral, methylprednisolone. This constellation of presentations, including chronic autoimmune hepatitis with mild cholangitis and pulmonary hypertension, suggested that the presence of serum ACA might indicate relentless visceral organ damage.