Scleroderma renal crisis |
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| Authors: | Marie Hudson Cybele Ghossein Virginia Steen |
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| Affiliation: | 1. Department of Neurology, Hôpitaux Universitaires de Strasbourg, France;2. INSERM U1119 Biopathologie de la Myéline, Neuroprotection et Stratégies Thérapeutique, Strasbourg France;3. Clinical Investigation Center, Hôpitaux Universitaires de Strasbourg, Strasbourg France;1. Laboratoire commun de Biologie et Génétique Moléculaires, Hôpital Saint-Antoine, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France;2. Sorbonne Université-Inserm UMRS_938, Centre de Recherche Saint-Antoine (CRSA), Paris 75012, France;1. Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy;2. Department of Internal Medicine, University of Pisa School of Medicine, Pisa, Italy |
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| Abstract: | 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. |
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