Bilateral coronary and renal artery stenosis in a case with limited scleroderma

A 65‐year‐old woman with long‐standing limited scleroderma over 15 years presented in January 2004 with scleroderma renal crisis with rising blood pressure, worsening of renal function and non‐ST segment acute coronary syndrome. The patient's renal function deteriorated upon receiving angiotensin‐converting enzyme (ACE) inhibitors, prompting coronary and renal angiography, which revealed bilateral coronary and renal artery stenosis. In scleroderma patients with renal crisis and showing deterioration of renal function, the possibility of renal artery stenosis should be excluded because ACE inhibitors in this situation carry risks for deterioration of kidney function.     

Scleroderma renal crisis sine scleroderma during pregnancy

Renal crisis is a serious complication of systemic sclerosis. Its occurrence prior to the development of skin sclerosis is exceedingly rare. We report a patient who developed acute renal failure during pregnancy. Renal biopsy showed features compatible with scleroderma renal crisis but typical cutaneous changes were only evident 2 months after the renal episode. The relationship between pregnancy, scleroderma activity and renal crisis is discussed.     

Medical Treatment of Hypertension and Renal Failure in Scleroderma

Abstract: Until recently acute renal failure in sclero-derma has been uniformly fatal. Since 1973 there have been several reports of successful treatment by peritoneal and haemodialysis and nephrectomy, sometimes followed by renal transplantation. During the past few years there have been several reports of patients managed successfully with drug treatment. The present paper reports on a patient with scleroderma who is clinically well with stable renal function with drug treatment three years after the onset of acute renal impairment and hypertension. Other similar cases are reviewed.     

Variable response to oral angiotensin-converting-enzyme blockade in hypertensive scleroderma patients

Twelve scleroderma patients with hypertension, seven of whom had malignant hypertension and renal failure of scleroderma renal crisis, were treated with captopril. The first dose lowered mean pressure in all patients by 21.3 mmHg; in 6 patients it relieved encephalopathy. Blood pressure was controlled in all patients. Two of 7 patients with scleroderma renal crisis had improvement in renal function; the 5 patients who did not have malignant hypertension improved or stabilized. Despite good pressure control, however, renal failure developed in 5 patients with scleroderma renal crisis. The data indicated that captopril is effective antihypertensive therapy in scleroderma and, when given early, may prevent renal failure and death.     

Atrial flutter, ventricular tachycardia and changing axis deviation associated with scleroderma

Rhythm disturbances have been described in immunological and connective diseases. Scleroderma is a fibrotic condition characterized by immunological abnormalities, vascular injury and increased accumulation of extracellular matrix proteins. The heart is one of the major organs involved in scleroderma, the involvement of which can be manifested by myocardial disease, conduction system abnormalities, arrhythmias, or pericardial disease. Additionally, scleroderma renal crisis and pulmonary hypertension lead to significant cardiac dysfunction secondary to damage in the kidney and lung. Changing axis deviation has been reported also during atrial fibrillation or atrial flutter. Changing axis deviation has been also reported during acute myocardial infarction associated with atrial fibrillation too or at the end of atrial fibrillation during acute myocardial infarction. We present a case of atrial flutter, ventricular tachycardia and changing axis deviation in a 61-year-old Italian woman with scleroderma. This case focuses attention on changing axis deviation and on the presentation of arrhythmias in scleroderma. The underlying arrhythmogenic mechanisms are probably multiple and intriguing, even though the myocardial fibrosis and immunological autoantibody-mediated mechanisms seem to play a pivotal role.     

Value of captopril in the treatment of systemic arterial and pulmonary hypertension with increased plasma renin in scleroderma

The effects of the converting enzyme inhibitor captopril (Lopril) were studied in a 53 year old woman with acute exacerbation of scleroderma. In addition to her chronic symptoms of Raynaud's syndrome, the patient presented with severe hypertension, cardiac failure and oligoanuria. Right heart catheterisation with a Swan-Ganz catheter confirmed the systemic hypertension with cardiac failure, and also demonstrated precapillary pulmonary hypertension with raised pulmonary arterial resistance. The organic renal failure was an indication for renal biopsy which showed segmental and focal fibrinoid necrosis with microthrombosis and chronic ischemic changes. Due to raised plasma renin activity, treatment with captopril was instituted, leading to a rapid normalisation of systemic and pulmonary hypertension, the regression of cardiac failure and a transient improvement in the Raynaud's syndrome. The renal failure did not improve and the patient had to undergo chronic hemodialysis. These spectacular initial results should be interpreted in the context of the poor prognosis of acute exacerbations of scleroderma despite the encouraging data published recently after well-controlled antihypertensive therapy.     

An unusual cause of acute renal failure in systemic sclerosis

BACKGROUND: Scleroderma renal crisis is one of the most life threatening complications of scleroderma. Enteric hyperoxaluria complicates extensive disease or resection of the small intestine in the presence of an intact colon, and is associated with calcium oxalate nephrolithiasis. This cause of renal failure may be underestimated and should be considered in all patients with malabsorption and renal failure. CASE REPORT: A 78 year old woman with systemic sclerosis affecting the bowel developed acute renal failure caused by oxalate nephropathy. RESULTS: The patient's renal failure improved on an oxalate free diet.     

The pathogenesis of fibrosis and renal disease in scleroderma: Recent insights from glomerulosclerosis

Acute and chronic renal diseases remain common complications of systemic sclerosis. Although treatment for acute scleroderma renal crisis may arrest the rapid progression of renal disease, many patients develop persistent renal dysfunction. Based on recent insights gained from progressive renal diseases of diverse etiologies, novel approaches to understanding the pathobiology of scleroderma renal disease may be applicable. Key factors involved in progression of renal disease include accumulation of extracellular matrix in the glomerular and tubulointerstitial compartments, epithelial to mesenchymal transformation, and vascular changes. The relevant factors mediating these events include the reninangiotensin system, the profibrotic growth factors, transforming growth factor-beta and connective tissue growth factor, and reactive oxygen species. Much of the molecular details of the role of these factors have been revealed and promise to alter the practice of therapy of progressive renal disease.     

Scleroderma renal crisis sine scleroderma in pregnancy: a case report

Scleroderma renal crisis has been documented as the presenting manifestation of systemic sclerosis sine scleroderma in pregnancy only once in the literature. Unfortunately, since scleroderma renal crisis in sine scleroderma pregnant patients is so rare, that patient expired. We present a case of a sine scleroderma pregnant patient with an initial manifestation of scleroderma renal crisis surviving due to successful diagnosis and treatment.     

Control of hypertension and reversal of renal failure in undifferentiated connective tissue disease by enalapril

Activation of the renin angiotensin system is important in the development of accelerated hypertension and progression to acute renal failure in scleroderma and undifferentiated connective tissue disease. Inhibition of angiotensin-converting enzyme activity may effectively control blood pressure and ameliorate renal insufficiency. To our knowledge, we describe the first reversal of dialysis-dependent renal insufficiency by enalapril maleate and recovery and maintenance of near-normal renal function in a patient suffering from undifferentiated connective tissue disease with sclerodermatous features. The pathophysiologic mechanisms and long-term treatment implications with angiotensin-converting enzyme inhibitors in this setting are discussed.     

Scleroderma and pregnancy. New case and review of the literature

A new case of the rare association of scleroderma and pregnancy is reported. The pregnancy was complicated by renal failure in the last month of gestation which was initially well controlled by anti-hypertensive therapy but then suddenly progressed to pre eclampsia with signs of foetal distress necessitating emergency caesarian section. A moderately hypotrophic child was delivered. The mother progressively recovered; diuresis and blood pressure returned to normal and the proteinuria disappeared. In the light of previously reported cases and of recent advances in our knowledge of scleroderma, especially scleroderma renal disease, the authors discuss their attitude to the management of women with scleroderma wishing to become pregnant. They review the role and place of renal biopsy in the detection of subclinical renal lesions due to the scleroderma, the presence of which would be a decisive factor in assessing the risks of pregnancy. Scleroderma renal disease is, in fact, a major contraindication to pregnancy because of the very poor foetal prognosis and the risk of maternal death due to a lethal progression of renal failure.     

Systemic sclerosis and antineutrophil cytoplasmic autoantibody-associated renal failure

We report three patients who developed antineutrophil cytoplasmic autoantibody (ANCA)-associated crescentic glomerulonephritis, two of whom showed clinical features of limited scleroderma and one whose results of serological tests were suggestive of limited scleroderma without cutaneous features. All had anticentromere antibodies and antimyeloperoxidase antibodies. No patient showed the features of typical scleroderma renal crisis such as accelerated hypertension or microangiopathy. Our patients were normotensive at the time of onset of renal failure, and the clinical picture was characterised by only modest features of limited scleroderma. All three patients had crescentic glomerulonephritis at various stages of chronicity. One patient responded to immunosuppressive therapy with improvement in renal function; the other two patients rapidly developed end-stage renal failure. These patients and others recently described may represent a newly described form of scleroderma renal disease. Received: 12 January 1999 / Accepted: 3 May 1999     

Factors predicting development of renal involvement in progressive systemic sclerosis

Renal involvement or "scleroderma renal crisis" developed in 60 patients with progressive systemic sclerosis evaluated at the University of Pittsburgh during the period from 1972 to 1982. Forty-seven of these patients had progressive systemic sclerosis with diffuse scleroderma, representing 18 percent of persons with progressive systemic sclerosis and diffuse scleroderma evaluated during this time period. Ten additional patients did not have truncal scleroderma but were suspected of having incompletely developed diffuse scleroderma. Only three patients were classified as having progressive systemic sclerosis with the CREST syndrome. Renal crisis was observed early in the course of the illness, a mean of 3.2 years after onset. During May and June, this complication developed in fewer patients than expected. Thirty-six patients who had diffuse scleroderma and renal involvement after their initial Pittsburgh evaluation were compared with 212 who had diffuse scleroderma without renal involvement during follow-up. The patients with renal involvement had a shorter mean disease duration at the time of their first evaluation (2.4 versus 4.2 years, p less than 0.05) and less frequently had digital pitting scars (29 versus 54 percent), but no other significant clinical, laboratory, or serologic differences were noted. Data available for 31 patients with renal involvement during the six months preceding the onset of renal disease were analyzed. Blood pressure, serum creatinine, urine protein and red blood cells, and plasma renin levels were similar in these patients and the 212 patients without renal involvement. More patients with renal involvement had anemia or clinical evidence of cardiac involvement during this period compared with the patients without renal involvement. During the 12-month period prior to renal involvement, seven of 16 (44 percent) patients with such involvement had an impressive increase in skin thickening on physical examination compared with only 23 of 180 (14 percent) patients without renal involvement at any time during their course. Thus, the subset of patients with diffuse scleroderma who show rapid progression of their skin thickening early in the illness with development of anemia, pericardial effusion, or congestive heart failure have a high risk of "scleroderma renal crisis."     

The changing face of severe scleroderma in five patients

Five cases from recent experience are reported to characterize the changing pattern of disease. With the introduction of potent antihypertensive agents, especially the angiotensin I-II converting enzyme inhibitors (CEI), the clinical pattern of scleroderma renal involvement has changed from an acute, oliguric, usually fatal renal failure to an indolent functional decline of the kidneys, heart and lungs together.     

Resolution of renal failure with malignant hypertension in scleroderma. Case report and review of the literature.

The ominous prognosis of rapidly progressive renal failure associated with malignant hypertension in scleroderma has led to aggressive management by dialysis, early bilateral nephrectomy and renal transplantation. We describe a woman with scleroderma who recovered after the development of malignant hypertension and renal failure. Renal biopsy and arteriography demonstrated the classic vascular lesions of scleroderma with secondary cortical ischemia. Of note, plasma renin activity was normal. Review of the literature revealed 40 patients with scleroderma and rapidly progressive renal failure who have been treated by dialysis. Thirteen patients survived, four of whom did not undergo bilateral nephrectomy. In eight patients treated by transplantation, five achieved excellent allograft function although one sustained a late rejection. Analysis of our case and of five recently reported cases of reversible renal failure and malignant hypertension reveals no distinctive features identifying patients with a favorable prognosis, except for the normal plasma renin activity level in our patient. Vigorous control of hypertension and renal failure by drugs and dialysis is recommended. Bilateral nephrectomy should be considered only for patients with refractory hypertension, since recovery of renal function may follow even after months of dialysis. For patients with irreversible renal failure without other major organ system involvement, transplantation is a reasonable alternative to dialysis.     

Scleroderma renal crisis

Abrupt onset of severe uncontrolled hypertension and rapidly progressive oliguric renal failure characterizes scleroderma renal crisis. The etiology is unclear, but very high renin levels are present. While scleroderma is more common in women and whites, there is no difference in the prevalence of scleroderma renal crisis by gender. However, there appears to be a higher prevalence of scleroderma renal crisis among African Americans than whites. Survival was dismal prior to the introduction of the vigorous treatment of hypertension and use of converting-enzyme inhibitors. However, most data on the benefit of these medications are derived from uncontrolled and unblinded studies. Prospective, randomized controlled trials are needed to assess the role of angiotensin receptor blockers. Prevention trials could define the role of various drugs in decreasing the rate of scleroderma renal crisis.     

Pulmonary hypertension confirmed histologically five months prior to scleroderma renal crisis onset

A 69-year-old man presented shortness of breath and acute renal failure. He had undergone pulmonary partial resection for lung cancer 5 months prior. On examination, severe hypertension, skin sclerosis of his forearms, and anticentromere antibody were observed. A renal biopsy specimen showed characteristic findings for scleroderma renal crisis, and a right heart catheterization revealed severe pulmonary arterial hypertension. Re-examination of the resected lung specimen revealed sclerodermatous vascular involvement was present.     

Long-term outcomes of scleroderma renal crisis

BACKGROUND: Although scleroderma renal crisis, a complication of systemic sclerosis, can be treated with angiotensin-converting enzyme (ACE) inhibitors, its long-term outcomes are not known. OBJECTIVE: To determine outcomes, natural history, and risk factors in patients with systemic sclerosis and scleroderma renal crisis. DESIGN: Prospective observational cohort study. SETTING: University program specializing in scleroderma. PATIENTS: 145 patients with scleroderma renal crisis who received ACE inhibitors and 662 patients with scleroderma who did not have renal crisis. MEASUREMENTS: Among patients with renal crisis, the four outcomes studied were no dialysis, temporary dialysis, permanent dialysis, and early death. Demographic, clinical, and laboratory data were compared to identify risk factors for specific outcomes. Follow-up was 5 to 10 years. RESULTS: 61% of patients with renal crisis had good outcomes (55 received no dialysis, and 34 received temporary dialysis); only 4 of these (4%) progressed to chronic renal failure and permanent dialysis. More than half of the patients who initially required dialysis could discontinue it 3 to 18 months later. Survival of patients in the good outcome group was similar to that of patients with diffuse scleroderma who did not have renal crisis. Some patients (39%) had bad outcomes (permanent dialysis or early death). CONCLUSIONS: Renal crisis can be effectively managed when hypertension is aggressively controlled with ACE inhibitors. Patients should continue taking ACE inhibitors even after beginning dialysis in hopes of discontinuing dialysis.     

Pulmonary hypertension confirmed histologically five months prior to scleroderma renal crisis onset

Abstract

A 69-year-old man presented shortness of breath and acute renal failure. He had undergone pulmonary partial resection for lung cancer 5 months prior. On examination, severe hypertension, skin sclerosis of his forearms, and anticentromere antibody were observed. A renal biopsy specimen showed characteristic findings for scleroderma renal crisis, and a right heart catheterization revealed severe pulmonary arterial hypertension. Re-examination of the resected lung specimen revealed sclerodermatous vascular involvement was present.     

系统性硬化症的肾脏表现

系统性硬化症肾脏受累的主要类型包括硬皮病肾危象、慢性肾疾病和炎症性肾损害,肾脏受累仍然是硬皮病的主要并发症之一。硬皮病肾危象需要早期诊断,积极使用血管紧张素转化酶抑制剂。尽管使用血管紧张素转化酶抑制剂,硬皮病肾危象患者的预后仍差。系统性硬化症患者中存在抗中性粒细胞胞浆抗体相关血管炎。任何没有高血压的硬皮病患者出现肾功能损害或明显蛋白尿应考虑行肾活检。