A merchant mariner had scleroderma with polyarthralgias and Raynaud's phenomenon for 15 years before the onset of rapidly progressive renal failure and severe hypertension. lntimal proliferation obliterated or greatly narrowed the lumina of renal arterioles. Initially, he tolerated periodic hemodialysis and efforts to reduce hypertension poorly but did better after bilateral nephrectomy. In the 18 months since successful kidney transplantation, arthralgias and Raynaud's phenomenon have disappeared and skin has become less tightly bound to underlying structures. Blood pressure remains normal. The grafted kidney shows no evidence of disease. For patients whose primary organ involvement by scleroderma is renal and whose other lesions are relatively stable, hemodialysis and kidney transplantation may provide additional years of reasonably comfortable life.Systemic sclerosis (scleroderma) is often associated with fulminating renal failure (1, 2). Changes in renal biopsies are indistinguishable from those of malignant hypertension even though the patient's blood pressure is not always greatly elevated (3). When renal failure occurs, the systemic nature of the primary disease ordinarily precludes expensive, scarce hemodialysis or kidney transplantation. We have successfully used both of these treatments in a man whose nonrenal manifestations of scleroderma seemed static and, in themselves, no bar to this therapy.
CASE REPORTA 41 -year-old merchant seaman first experienced stiffness and aching of various joints in 1955. Early in 1958, at age 27, he noticed progressive tightening and thickening of the skin of his hands, and to a lesser extent, his forearms, face and feet. Histologic examination of a specimen of skin from a finger showed atrophy of the epidermis and appendages with swelling, hypertrophy and condensation of the collagen in the dermis. During his first hospitalization in October 1958, digitalis was prescribed for presumed mild congestive heart failure, manifested by bibasilar pulmonary rales and shortness of breath. The rales and dyspnea disappeared. At that time, blood pressure, chest x-ray, electrocardiogram, urinalysis, rate of phenolsulfonphthalein excretion, a Fishberg urine concentration test, serum creatinine and a renal biopsy all were normal.He was evaluated again in 1962. Skin changes showed no obvious progression. He complained of generalized aching in his joints and frequent attacks of painful blanching of his fingers in cold weather. Blood pressures, urinalyses and tests of renal function remained normal, as did a second renal biopsy. Agglutination tests for rheumatoid factor and tests for lupus erythematosus cells were negative. The digitalis was stopped without ill effect.In 1968
RICHARDSONwere normal. Raynaud's phenomenon and polyarthralgias had persisted over the years, and he had, more or less, adapted to them. Finger tips were sharply tapered. Shiny skin of hands and fingers, tightly hound to underlying structures, prevented a complete grip. Similar, hut less extensive, changes involved...