PubMed2002
HISTORY AND CLINICAL FINDINGS: Since several years, two women, 69 and 75 years of age, developed a paroxysmal blanching of the fingers on both hands. The attacks were accompanied by local pain and followed by a short period of cyanotic discoloration. INVESTIGATIONS: The diagnosis of progressive systemic sclerosis was reached by skin biopsies, immunoserological profile and significant blood vessel alteration at the proximal nail fold (capillary microscopy) in both patients. Initial esophageal sclerosis was only found in one patient. Both women were conspicuous by sonographically proven hepatomegaly, elevated hepatic serological parameters, in particular aP and GGTP. Positive AMA titers of M2 specificity and specific features in liver biopsies yielded the additional diagnosis of primary biliary cirrhosis). TREATMENT AND COURSE: One of the women was treated by pentoxifylline and nifedipine in order to improve rheology. Both received physiotherapeutic assistance. Primary biliary cirrhosis was treated with ursodeoxycholic acid which led to normalization of the cholestatic parameters. CONCLUSIONS: Reynolds' syndrome constitutes the coexistence of progressive systemic sclerosis and primary biliary cirrhosis. It remains unknown whether or not there is a constant time-related sequence of the clinical manifestations of the two immunogenetically determined disorders. However, it is remarkable that the affected elderly women described so far had Raynaud's symptoms long before the final diagnosis of Reynolds' syndrome was settled. Thus, the Raynaud's phenomenon is an early clinical symptom for the evolution of a Reynolds' syndrome. By experience, the coincidence of progressive systemic sclerosis and primary biliary cirrhosis seems to be a favorable factor concerning the progression of primary biliary cirrhosis. So, an elderly woman with recently manifested progressive systemic sclerosis should alert the physician to a concomitant primary biliary cirrhosis.