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Renal complications and scleroderma renal crisis.
Rheumatology (Oxford). 2009 Jun; 48 Suppl 3:iii32-5.R

Abstract

Scleroderma renal crisis (SRC) occurs in 5-10% of SSc patients, who may present with an abrupt onset of hypertension, acute renal failure, headaches, fevers, malaise, hypertensive retinopathy, encephalopathy and pulmonary oedema. Patients at greatest risk of developing SRC are those with diffuse cutaneous or rapidly progressive forms of SSc, and treatment with a recently commenced high dose of corticosteroid. Laboratory tests may demonstrate hypercreatinaemia, microangiopathic haemolytic anaemia (MAHA), thrombocytopaenia and hyperreninaemia. Renal crisis is also linked to a positive ANA speckled pattern, antibodies to RNA polymerase I and II, and an absence of anti-centromere antibodies. Early, aggressive treatment with angiotensin-converting enzyme inhibitors has improved prognosis in SRC, although 40% of the patients may require dialysis, and mortality at 5 yrs is 30-40%. Median time to recovery is 1 yr, and typically occurs within 3 yrs. Prognosis is worse for males, but may not be related to corticosteroid use, presence of MAHA or severity of renal pathology. Modification of endothelin over-activity, which is implicated in the pathogenesis of SRC, may offer a future therapeutic approach.

Authors+Show Affiliations

Centre for Rheumatology, Royal Free Hospital, Rheumatology Unit, London, UK. c.denton@medsch.ucl.ac.ukNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

19487221

Citation

Denton, C P., et al. "Renal Complications and Scleroderma Renal Crisis." Rheumatology (Oxford, England), vol. 48 Suppl 3, 2009, pp. iii32-5.
Denton CP, Lapadula G, Mouthon L, et al. Renal complications and scleroderma renal crisis. Rheumatology (Oxford). 2009;48 Suppl 3:iii32-5.
Denton, C. P., Lapadula, G., Mouthon, L., & Müller-Ladner, U. (2009). Renal complications and scleroderma renal crisis. Rheumatology (Oxford, England), 48 Suppl 3, iii32-5. https://doi.org/10.1093/rheumatology/ken483
Denton CP, et al. Renal Complications and Scleroderma Renal Crisis. Rheumatology (Oxford). 2009;48 Suppl 3:iii32-5. PubMed PMID: 19487221.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Renal complications and scleroderma renal crisis. AU - Denton,C P, AU - Lapadula,G, AU - Mouthon,L, AU - Müller-Ladner,U, PY - 2009/6/3/entrez PY - 2009/6/12/pubmed PY - 2009/7/7/medline SP - iii32 EP - 5 JF - Rheumatology (Oxford, England) JO - Rheumatology (Oxford) VL - 48 Suppl 3 N2 - Scleroderma renal crisis (SRC) occurs in 5-10% of SSc patients, who may present with an abrupt onset of hypertension, acute renal failure, headaches, fevers, malaise, hypertensive retinopathy, encephalopathy and pulmonary oedema. Patients at greatest risk of developing SRC are those with diffuse cutaneous or rapidly progressive forms of SSc, and treatment with a recently commenced high dose of corticosteroid. Laboratory tests may demonstrate hypercreatinaemia, microangiopathic haemolytic anaemia (MAHA), thrombocytopaenia and hyperreninaemia. Renal crisis is also linked to a positive ANA speckled pattern, antibodies to RNA polymerase I and II, and an absence of anti-centromere antibodies. Early, aggressive treatment with angiotensin-converting enzyme inhibitors has improved prognosis in SRC, although 40% of the patients may require dialysis, and mortality at 5 yrs is 30-40%. Median time to recovery is 1 yr, and typically occurs within 3 yrs. Prognosis is worse for males, but may not be related to corticosteroid use, presence of MAHA or severity of renal pathology. Modification of endothelin over-activity, which is implicated in the pathogenesis of SRC, may offer a future therapeutic approach. SN - 1462-0332 UR - https://www.unboundmedicine.com/medline/citation/19487221/Renal_complications_and_scleroderma_renal_crisis_ L2 - https://academic.oup.com/rheumatology/article-lookup/doi/10.1093/rheumatology/ken483 DB - PRIME DP - Unbound Medicine ER -