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

Abstract

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 calcifi cations, digital ulcers and facial telangiectasias. Chest radiography showed signs of lung fi brosis. 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.

Patrón colestásico y enfermedad autoinmune: Una relación suspicaz

“…9 La co-ocurrencia de estas dos entidades se conoce como síndrome de Reynolds. 10 El diagnóstico se basa en la evaluación por separado de cada patología, tomando en cuenta que el 1 -2,5 % de los pacientes con esclerodermia tienen CBP y al contrario el 5-15% con CBP tienen esclerodermia. [11][12] Se presenta principalmente en población femenina (relación 10:1), entre la quinta y sexta década de vida.…”

“…10 El diagnóstico se basa en la evaluación por separado de cada patología, tomando en cuenta que el 1 -2,5 % de los pacientes con esclerodermia tienen CBP y al contrario el 5-15% con CBP tienen esclerodermia. [11][12] Se presenta principalmente en población femenina (relación 10:1), entre la quinta y sexta década de vida. 13 En más del 50% de casos, hepática de 15.1 kpa con IQR de 8,4 % (confiable) con score METAVIR F4 compatible con cirrosis.…”