Hepatitis, cholangitis, pulmonary hypertension, digital gangrene, and conjunctivitis sicca in a woman with anticentromere antibodies.
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.
Tsai
Chang-Youh
CY
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
Huo
An-Ping
AP
Chou
Chung-Tei
CT
Wu
Tsai-Hung
TH
Tsai
Ying-Yang
YY