Scleroderma renal crisis as an initial presentation of systemic sclerosis: a case report and review of the literature
K.M. Logee1, S. Lakshminarayanan2
- Division of Rheumatology, University of Connecticut Health Center, Farmington, Connecticut, USA. firstname.lastname@example.org
- Division of Rheumatology, University of Connecticut Health Center, Farmington, Connecticut, USA.
2015 Vol.33, N°4 ,Suppl.91
PI 0171, PF 0174
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PMID: 25797912 [PubMed]
Accepted : 24/11/2014
In Press: 06/03/2015
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.