title: Scleroderma renal crisis as an initial presentation of systemic sclerosis: a case report and review of the literature.
abstract: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) that is characterised by new-onset malignant hypertension and progressive acute renal failure, often with associated microangiopathic haemolytic anaemia and thrombocytopenia. SRC was at one time almost uniformly fatal, with death often occurring within a few weeks. With the development of angiotensin-converting-enzyme inhibitors (ACE-I), survival has improved dramatically, but death rates still remain unacceptably high. About 20% of SRC cases occur prior to making a diagnosis of SSc and, in some cases, there is no evidence of skin sclerosis at the time that SRC develops. In this report, we present a case in which a patient developed SRC prior to being diagnosed with scleroderma. Additionally, we review the pathogenesis, presenting signs and symptoms, management and prognosis of SRC.
authors: Division of Rheumatology, University of Connecticut Health Center, Farmington, Connecticut, USA. logee@uchc.edu.
authors: Kristin M
authors: KM
authors: Logee
authors: Division of Rheumatology, University of Connecticut Health Center, Farmington, Connecticut, USA.
authors: Santhanam
authors: S
authors: Lakshminarayanan
mesh_terms: D058186:Acute Kidney Injury / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D000743:Anemia, Hemolytic / Q000175:diagnosis / Q000209:etiology; D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D002216:Captopril / Q000627:therapeutic use; D057210:Delayed Diagnosis; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D006974:Hypertension, Malignant / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D008875:Middle Aged; D011237:Predictive Value of Tests; D006435:Renal Dialysis; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013777:Tetrazoles / Q000627:therapeutic use; D013921:Thrombocytopenia / Q000175:diagnosis / Q000209:etiology; D013997:Time Factors; D016896:Treatment Outcome; D014633:Valine / Q000031:analogs & derivatives / Q000627:therapeutic use; D000068756:Valsartan
chemical_list: D047228:Angiotensin II Type 1 Receptor Blockers; D000806:Angiotensin-Converting Enzyme Inhibitors; D000959:Antihypertensive Agents; D013777:Tetrazoles; D000068756:Valsartan; D002216:Captopril; D014633:Valine