Renal transplantation for progressive systemic sclerosis with renal failure

Merino, G E; Sutherland, David E.R.; Kjellstrand, Carl M.; Simmons, Richard L.; Najarían, John S.

doi:10.1016/0002-9610(77)90170-2

Cited by 34 publications

(11 citation statements)

References 7 publications

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“…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.…”

Section: Discussionsupporting

confidence: 86%

“…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].…”

Section: Discussionmentioning

confidence: 99%

“…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].…”

Section: Discussionmentioning

confidence: 99%

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Continuous Ambulatory Peritoneal Dialysis and Scleroderma

Copley¹,

Smith²

1985

Nephron

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A patient with scleroderma has been maintained on continuous ambulatory peritoneal dialysis (CAPD) for the past 18 months after having developed associated renal failure. Peritoneal clearances, pulmonary function tests, and hand studies have shown seasonal changes from summer to winter with ambient temperature variations. Rehabilitation and quality of life have been acceptable, blood pressure has been well controlled off all medications, and there has been relatively little disease progression. Attempts have been made to maintain low-dose captopril therapy to suppress plasma renin activity. This case suggests that CAPD is an acceptable and, perhaps, indicated treatment modality for renal failure associated with scleroderma.

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Section: Discussionsupporting

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Continuous Ambulatory Peritoneal Dialysis and Scleroderma

Copley¹,

Smith²

1985

Nephron

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“…Nevertheless, renal transplantation is the treatment of choice for most patients with ESRD due to scleroderma. Although most patients with scleroderma and ESRD do well after renal transplantation, approximately 20% may have a recurrence in the allograft, and many of these will develop graft failure as a result (453)(454)(455). In a report from the UNOS Scientific Registry, 86 patients with scleroderma were transplanted over a 10-year period from 1987 to 1997 (456).…”

Section: The Evaluation Of Renal Transplant Candidates: Clinical Pracmentioning

confidence: 99%

“…Graft loss from recurrence often occurred in patients in whom the time interval from onset of the disease to transplantation was less than 1 year, but this experience accrued prior to the CsA era (454). Patients who have undergone bilateral nephrectomy, often to control severe hypertension, may have the best survival (261).…”

Section: The Evaluation Of Renal Transplant Candidates: Clinical Pracmentioning

confidence: 99%

The Evaluation of the Renal Transplant Candidates: Clinical Practice Guidelines

2001

American Journal of Transplantation

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

Markenson

Sherman

1979

Arthritis & Rheumatism

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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...

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Renal transplantation for progressive systemic sclerosis with renal failure

Cited by 34 publications

References 7 publications

Continuous Ambulatory Peritoneal Dialysis and Scleroderma

Continuous Ambulatory Peritoneal Dialysis and Scleroderma

The Evaluation of the Renal Transplant Candidates: Clinical Practice Guidelines

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

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