Renal involvement in progressive systemic sclerosis: Prolonged survival with aggressive antihypertensive management

Abstract

The 7-year survival rate of patients with progressive systemic sclerosis (PSS; scleroderma) without kidney, heart, or lung involvement is reported as 56% (1). A significantly decreased survival for patients with cardiopulmonary and renal involvement has been reported (1) and it is unusual for patients to live beyond 6 months when the kidney is affected (2). Recognition of hyperreninemia (3) in the renal disease of scleroderma has prompted treatment with specific anti-renin agents. We report a case of hypertensive renal disease in PSS that was recognized as high renin hypertension and treated aggressively. Although severe renal dysfunction ensued, the patient's course eventually stabilized and she remains alive and ambulatory 5 years later.Case report. A 42-year-old black woman was admitted to the Hospital for Special Surgery at age 37 with a 10-month history of bilateral swelling of all proximal interphalangeal and metacarpophalangeal joints, hyperpigmentation of the chest, back, and abdomen, and a 4-month history of tightening of the skin over her forearms, abdomen, and thighs. A 25-pound weight loss had been noted over the preceding 10 months. Difficulty in swallowing solids, but not liquids, and anterior sternal chest pain after eating had been present for 1 month. Pain in her fingertips, followed by cyanosis, occurred after exposure to cold. Multiple blood pressure readings of 120/80 were recorded 3 years before onset of disease and readings of 130/100 were noted 9 months prior to her first clinic visit. Initial evaluation at the Hospital for Special Surgery revealed a pleasant black woman with definite atrophy and tight skin over the anterior chest, legs, forearms, thighs, abdomen, and distal lingers. There were multiple infarcts of the hands and areas of hyperpigmentation around the nail beds and splinter hemorrhages. Blood pressure was 180/110 in both arms when the patient was standing and sitting. Eye grounds demonstrated arteriolar narrowing. Examination of the chest revealed vesicular breath sounds without rales, rhonchi, or wheezes. Cardiac examination revealed a normal-sized heart, normal first heart sound, accentuation of the pulmonic component of the second heart sound, and a grade 2/4 systolic ejection murmur heard best along the left sternal border without radiation to the neck or axilla. There was no evidence of edema. Examination of the joints revealed swelling of all proximal interphalangeal and metacarpophalangeal joints with normal range of motion of hips, elbows, shoulders, wrists, knees, ankles, and spine.The diagnosis of PSS was confirmed on skin biopsy taken from a tight hyperpigmented area of the lower abdomen, which showed hypertrophy of collagen bundles, decreased numbers of fibroblasts, and atrophy of sweat glands. Laboratory data included: hematocrit of 37; normal urinalysis; BUN of 12 mg%, and creatinine of 1.0 mg%. Creatinine clearance was 74 ml/minUte/ 1.73 mm2 body surface area. Initial peripheral venous blood plasma renin was 2.65 nanograms/ml (normal 0.13-0.93) with a repeat v...