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Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc) characterized by malignant hypertension and acute kidney injury. Historically, SRC was the leading cause of death in SSc. However, with the advent of angiotensin converting enzyme (ACE) inhibitors, mortality rates have decreased significantly. Nevertheless, one-year outcomes remain poor, with over 30% mortality and 25% of patients remaining dialysis-dependent. There is an urgent need to improve early recognition and treatment, and to identify novel treatments to improve outcomes of SRC. In this chapter, the clinical features, classification, pathophysiology, differential diagnosis, management and outcomes of SRC are presented. Specific issues relating to pregnancy, prophylactic ACE inhibition and management of essential hypertension are also discussed., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Objective: The prognosis of patients with hypertensive emergencies has recently improved dramatically owing to the development of effective antihypertensive therapy. We examined the histological and clinical features of patients with hypertensive emergency-related nephropathy., Methods: Twelve patients (11 men and one woman) were diagnosed as having hypertensive emergencies with acute renal failure according to the Joint National Committee-7 classification of blood pressure for adults and underwent renal biopsies at our hospital between 1995 and 2008. These patients were enrolled in this retrospective study., Results: The age of the subjects was 40.1±9.8 years. At presentation, the mean systolic/diastolic blood pressure was 232±32/146±12 mmHg and none of the patients were being treated with antihypertensive drugs, although 10 patients had histories of hypertension. The mean serum creatinine level was 6.1±4.7 mg/dL. All 12 patients showed left ventricular hypertrophy on echocardiography. On light microscopy of the renal biopsy specimens, all 12 patients showed onion skin patterns of the arterioles; however, no fibrinoid necrosis of the small arteries was found. Electron microscopy revealed electron-lucent widening of the subendothelial zone of the glomerular capillary walls in seven patients. One of the 12 patients did not respond to medical therapy and required regular dialysis. The other 11 patients responded to treatment., Conclusion: An onion skin pattern of the arterioles is the most frequent histological finding in patients with hypertensive emergency-related nephropathy. Long-standing hypertension might contribute to this arteriolar change, since left ventricular hypertrophy was also seen in these patients. With strict control of hypertension using antihypertensive medications, the prognosis of patients with hypertensive emergency-related nephropathy can be improved.

We report a series of seven patients who had scleroderma renal crisis. Their primary clinical and laboratory features along with the details of their management were compared with those of similar cases from the literature. The seven patients died within one to four months of the diagnosis with a pattern of acute renal failure, left ventricular failure and malignant hypertension. Histopathologic examination was performed in four of the patients, in two of whom it revealed thickening of the wall of the interlobular arteries related to the scleroderma, and in the other two patients nonspecific lesions of malignant hypertension. This histopathologic particularity led us to propose, on the basis of multiple renal biopsies performed in patients with scleroderma, a lesion chronology of the kidney in patients with scleroderma. Nevertheless, the triggering factors and pathophysiologic mechanisms of scleroderma renal crisis remain unclear and its prognosis is severe. Early treatment with angiotensin-converting enzyme inhibitors and other vasodilatators administered intravenously can prevent death and dialysis.