1. Renal involvement in progressive systemic sclerosis is characterized by hypertension, grade III or IV hypertensive retinopathy, rapidly progressive renal failure and enhanced plasma renin activity.
2. Of 70 patients with progressive systemic sclerosis involving the kidneys seen since 1955, 48 died within less than 3 months and 16 are alive or survived longer than 1 year.
3. Aggressive antihypertensive therapy was the principal factor that prevented early death in the 16 survivors.
4. Angiotensin converting enzyme inhibition with captopril appears to be the treatment of choice at present, but methyldopa or minoxidil may be successful in certain patients whose response to captopril is not satisfactory.