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Scleroderma renal crisis as an initial presentation of systemic sclerosis: a case report and review of the literature.
Clin Exp Rheumatol. 2015 Jul-Aug; 33(4 Suppl 91):S171-4.CE

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+Show Affiliations

Division of Rheumatology, University of Connecticut Health Center, Farmington, Connecticut, USA. logee@uchc.edu.Division of Rheumatology, University of Connecticut Health Center, Farmington, Connecticut, USA.

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

25797912

Citation

Logee, Kristin M., and Santhanam Lakshminarayanan. "Scleroderma Renal Crisis as an Initial Presentation of Systemic Sclerosis: a Case Report and Review of the Literature." Clinical and Experimental Rheumatology, vol. 33, no. 4 Suppl 91, 2015, pp. S171-4.
Logee KM, Lakshminarayanan S. Scleroderma renal crisis as an initial presentation of systemic sclerosis: a case report and review of the literature. Clin Exp Rheumatol. 2015;33(4 Suppl 91):S171-4.
Logee, K. M., & Lakshminarayanan, S. (2015). Scleroderma renal crisis as an initial presentation of systemic sclerosis: a case report and review of the literature. Clinical and Experimental Rheumatology, 33(4 Suppl 91), S171-4.
Logee KM, Lakshminarayanan S. Scleroderma Renal Crisis as an Initial Presentation of Systemic Sclerosis: a Case Report and Review of the Literature. Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S171-4. PubMed PMID: 25797912.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Scleroderma renal crisis as an initial presentation of systemic sclerosis: a case report and review of the literature. AU - Logee,Kristin M, AU - Lakshminarayanan,Santhanam, Y1 - 2015/03/06/ PY - 2014/03/21/received PY - 2014/11/24/accepted PY - 2015/3/24/entrez PY - 2015/3/24/pubmed PY - 2015/10/27/medline SP - S171 EP - 4 JF - Clinical and experimental rheumatology JO - Clin. Exp. Rheumatol. VL - 33 IS - 4 Suppl 91 N2 - 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. SN - 0392-856X UR - https://www.unboundmedicine.com/medline/citation/25797912/Scleroderma_renal_crisis_as_an_initial_presentation_of_systemic_sclerosis:_a_case_report_and_review_of_the_literature_ L2 - http://www.clinexprheumatol.org/pubmed/find-pii.asp?pii=25797912 DB - PRIME DP - Unbound Medicine ER -