Reynolds syndrome

Overview

Reynolds syndrome is a rare autoimmune disease, consisting of the combination of primary biliary cirrhosis and progressive systemic sclerosis. In some patients this syndrome has also been associated with Sjögren's syndrome and hemolytic anemia. Typical clinical features include jaundice, elevated blood levels of alkaline phosphatase, calcinosis cutis, telangiectasias, and pruritus. Raynaud's phenomenon may be an early clinical feature. The syndrome, a special case of scleroderma, is named after the American physician, Telfer B. Reynolds, MD (1921-2004), who first described it. He is also known for creating one of the world's first hepatology programs at the University of Southern California.

Symptoms

Hardened skin Liver cirrhosis Calcinosis cutis Raynaud's phenomenon Reduced esophageal motility

Diagnosis

In the last decade, with the aid of labial salivary gland biopsies, more patients with Reynold’s syndrome have been reported in the medical literature. In addition, there have been several reports of patients with Reynold’s syndrome who also developed other autoimmune conditions, usually Sjogren’s syndrome. In most cases, the symptoms of liver disease caused by primary biliary cirrhosis preceded the symptoms of systemic sclerosis. However, in most cases Raynaud’s syndrome was observed years before the symptoms of liver disease. Raynaud’s phenomenon, which can be a benign condition, is often an early finding in patients with systemic sclerosis.