Hemodialysis and kidney transplantation for renal failure from scleroderma

Abstract

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...

Continuous Ambulatory Peritoneal Dialysis and Scleroderma

“…In the latter report, blood pressure was easily controlled in both patients (1 without medications): 1 patient experienced improvement in sys temic scleroderma symptoms as well. Such improvement of systemic symptomatology has also been reported with other ESRD treatment modalities [3,5,6], CAPD has now been successfully accomplished in our patient for 18 months. Rehabilitation and quality of life have been acceptable to both her and her family.…”

“…While this control may be secondary to improved volume main tenance, it also may relate to removal of a dialyzable factor that contributes to hypertension. Such good con trol has not often been obtainable with either hemodialy sis or transplantation, and reports of bilateral nephrec tomy are not uncommon accompaniments to such treat ment modalities [1,[5][6][7]13]. It has now been well docu mented that renal function may improve when hyperten sion is controlled even months after such control is ob tained and despite the need for interim dialysis [7,12,13].…”

“…Hemodialysis has been used successfully for more than 12 months [3]. Potential problems pertain to difficulty in placing and maintaining vascular access to include needle placement into the access [4,5], as well as hemodynamic instability, especially if the heart or pericardium is involved. Trans plantation has also been used, but results are variable, complications high, and disease recurrence probably documented in the grafted kidney [5][6][7].…”

“…Potential problems pertain to difficulty in placing and maintaining vascular access to include needle placement into the access [4,5], as well as hemodynamic instability, especially if the heart or pericardium is involved. Trans plantation has also been used, but results are variable, complications high, and disease recurrence probably documented in the grafted kidney [5][6][7]. Finally, perito neal dialysis experience is sparse, possibly due to reports which show reduced peritoneal clearances of urea and creatinine in this disease state [8,9].…”

The Kidney in Scleroderma

“…1973 andDecember, 1975, 84 patients with diffuse scleroderma were seen at the Columbia Presbyterian Medical Center (CPMC). 57 of these patients were first seen during these 3 years; the remaining 27 who were being followed at the be ginning of the survey have been reported previously [ I ].…”