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 pruritis 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.