A Case of Reynolds Syndrome: Scleroderma and Primary Biliary Cirrhosis Overlap Syndrome

Marco Simoes, Patricia Alves, Helder Esperto, Benilde Barbosa, Lelita Santos, Jose Manuel Nascimento Costa


     Primary biliary cirrhosis (PBC) is a chronic liver condition characterized by bile duct destruction, probably caused by a yet unknown immunological mechanism. Its association with several other autoimmune diseases has been described. The association of the limited cutaneous form of systemic scleroderma (lcSSc) and PBC is known as Reynolds syndrome. The authors describe a case of a 76 year old male patient with Raynaud’s phenomenon complaints in 1999. The physical examination revealed sclerodactyly, cutaneous calcifications, digital ulcers and facial telangiectasias. Chest radiography showed signs of lung fibrosis. Dilated capillary loop were found in nailfold capillaroscopy. The autoimmunity pattern was compatible with limited systemic sclerosis (antinuclear and anticentromere antibodies were positive). In 2005 the autoimmunity pattern changed, with positive antimitochondrial antibodies. The patient presented with fatigue, pruritus and dyspnea on exertion complaints. Liver function tests showed elevation of alkaline phosphatase and gamma-glutamyl transpeptidase. Although the patient refused liver biopsy, PBC diagnosis was established. In 2008 the patient was admitted to the hospital with anorexia, dyspepsia, ascites, abdominal pain and weight loss. Endoscopy showed esophageal varices and abdominal computed tomography revealed cirrhotic liver. The patient died in 2009 with acute myocardial infarction.



Limited systemic cutaneous sclerosis; CREST syndrome; Primary biliary cirrhosis; Overlap syndrome; Reynolds syndrome; Autoimmunity; Nailfold capillaroscopy; Raynaud’s phenomenon

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