title: [Scleroderma renal crisis].
abstract: Scleroderma renal crisis (SRC) occurs in patients with systemic sclerosis (SSc) and is defined by otherwise unexplained rapidly progressive renal insufficiency associated with oliguria or rapidly progressive arterial hypertension or both. SRC is a rare and severe complication of SSc, most often encountered during the first 4 years of disease, almost only in patients with diffuse SSc. Factors predicting SRC were identified, including high-dose corticosteroid administration. Use of angiotensin-converting enzyme inhibitors (ACEI) has dramatically impressed the prognosis of SRC, but it mortality rate is still high. Treatment aims at normalizing blood pressure as soon as possible. ACEI should always be used, and additional anti-hypertensive agents, including calcium channel blockers and alpha- and beta-blockers, may be useful. Renal replacement therapy may be needed, but often (for almost half of patients) only temporarily.
authors.affiliation: Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour les Vascularites Nécrosantes et la Sclérodermie Systémique, Assistance Publique-Hôpitaux de Paris (AP-HP) et Université Paris-Descartes, Paris.
authors.forename: Luis
authors.initials: L
authors.lastname: Teixeira
authors.forename: Amélie
authors.initials: A
authors.lastname: Servettaz
authors.forename: Marion
authors.initials: M
authors.lastname: Mehrenberger
authors.forename: Laure-Hélène
authors.initials: LH
authors.lastname: Noël
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
mesh_terms: D058186:Acute Kidney Injury / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D000317:Adrenergic alpha-Antagonists / Q000627:therapeutic use; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D001706:Biopsy; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D002216:Captopril / Q000627:therapeutic use; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis; D006977:Hypertension, Renal / Q000175:diagnosis; D007668:Kidney / Q000473:pathology; D008297:Male; D009846:Oliguria / Q000209:etiology; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis; D011379:Prognosis; D017582:Renal Replacement Therapy; D012306:Risk; D012307:Risk Factors; D045743:Scleroderma, Diffuse / Q000150:complications; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D013997:Time Factors; D018608:Ultrasonography, Doppler
chemical_list: D000317:Adrenergic alpha-Antagonists; D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D002216:Captopril