Reynold's Syndrome

Posted by Elaine Moore Apr 13, 2007

Several autoimmune diseases are known to exist together as syndromes, for instance the polyglandular autoimmune syndromes. Another syndrome in which multiple autoimmune disorders co-exist is Reynold’s syndrome. Reynolds syndrome is a condition in which patients have progressive systemic sclerosis and the autoimmune liver disease primary biliary cirrhosis. Reynold’s syndrome is named after the American physician who first reported the association between the two disorders, Telfer B. Reynolds.

Who is Affected?

Reynold’s syndrome occurs primarily in women although it has been reported in men. It is usually diagnosed in the 6th to 7th decade of life although early symptoms may occur years before diagnosis.

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.

Associated Diseases

Besides its frequent association with Sjogren's syndrome, several reports in the literature describe an association between Reynold’s syndrome and the lupus erythematosus/lichen planus overlap syndrome. Recently, a patient was described who had Reynold’s syndrome and lupus erythematosus/lichen planus overlap syndrome as well as euthyroid (normal thyroid function) Hashimoto’s thyroiditis, diagnosed by the presence of thyroid antibodies. The findings in this patient correspond to type 2 of the multiple autoimmune syndrome.

In other reports, Reynold’s syndrome has been observed in patients with autoimmune hemolytic anemia and in patients with features of CREST syndrome, a condition which can occur in systemic sclerosis. Patients with Reynold’s syndrome often have acral scleroderma (involving the toes).

Resources:

Launay D, Hebbar M, Janin A, Primary biliary cirrhosis and systemic scleroderma (Reynolds syndrome): apropos of 8 new cases. The contribution of accessory salivary gland biopsy, Rev Med Interne June, 1998 19(6): 393-398.