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Scleroderma renal crisis: a rare but severe complication of systemic sclerosis.
Clin Rev Allergy Immunol. 2011 Apr; 40(2):84-91.CR

Abstract

Scleroderma renal crisis (SRC) is a major complication in patients with systemic sclerosis (SSc). It is characterized by malignant hypertension and oligo/anuric acute renal failure. SRC occurs in 5% of patients with SSc, particularly in the first years of disease evolution and in the diffuse form. The occurrence of SRC is more common in patients treated with glucocorticoids, the risk increasing with increasing dose. Left ventricular insufficiency and hypertensive encephalopathy are typical clinical features. Thrombotic microangiopathy is detected in 43% of the cases. Anti-RNA-polymerase III antibodies are present in one third of patients who develop SRC. Renal biopsy is not necessary if SRC presents with classical features. However, it can help to define prognosis and guide treatment in atypical forms. The prognosis of SRC has dramatically improved with the introduction of angiotensin-converting enzyme inhibitors (ACEi). However, 5 years survival in SSc patients who develop the full picture of SRC remains low (65%). SRC is often triggered by nephrotoxic drugs and/or intravascular volume depletion. The treatment of SRC relies on aggressive control of blood pressure with ACEi, if needed in combination with other types of antihypertensive drugs. Dialysis is frequently indicated, but can be stopped in approximately half of patients, mainly in those for whom a perfect control of blood pressure is obtained. Patients who need dialysis for more than 2 years qualify for renal transplantation.

Authors+Show Affiliations

UPRES EA, Pôle de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hospital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, France. luc.mouthon@cch.aphp.frNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

20012923

Citation

Mouthon, Luc, et al. "Scleroderma Renal Crisis: a Rare but Severe Complication of Systemic Sclerosis." Clinical Reviews in Allergy & Immunology, vol. 40, no. 2, 2011, pp. 84-91.
Mouthon L, Bérezné A, Bussone G, et al. Scleroderma renal crisis: a rare but severe complication of systemic sclerosis. Clin Rev Allergy Immunol. 2011;40(2):84-91.
Mouthon, L., Bérezné, A., Bussone, G., Noël, L. H., Villiger, P. M., & Guillevin, L. (2011). Scleroderma renal crisis: a rare but severe complication of systemic sclerosis. Clinical Reviews in Allergy & Immunology, 40(2), 84-91. https://doi.org/10.1007/s12016-009-8191-5
Mouthon L, et al. Scleroderma Renal Crisis: a Rare but Severe Complication of Systemic Sclerosis. Clin Rev Allergy Immunol. 2011;40(2):84-91. PubMed PMID: 20012923.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Scleroderma renal crisis: a rare but severe complication of systemic sclerosis. AU - Mouthon,Luc, AU - Bérezné,Alice, AU - Bussone,Guillaume, AU - Noël,Laure-Hélène, AU - Villiger,Peter M, AU - Guillevin,Loïc, PY - 2009/12/17/entrez PY - 2009/12/17/pubmed PY - 2011/8/6/medline SP - 84 EP - 91 JF - Clinical reviews in allergy & immunology JO - Clin Rev Allergy Immunol VL - 40 IS - 2 N2 - Scleroderma renal crisis (SRC) is a major complication in patients with systemic sclerosis (SSc). It is characterized by malignant hypertension and oligo/anuric acute renal failure. SRC occurs in 5% of patients with SSc, particularly in the first years of disease evolution and in the diffuse form. The occurrence of SRC is more common in patients treated with glucocorticoids, the risk increasing with increasing dose. Left ventricular insufficiency and hypertensive encephalopathy are typical clinical features. Thrombotic microangiopathy is detected in 43% of the cases. Anti-RNA-polymerase III antibodies are present in one third of patients who develop SRC. Renal biopsy is not necessary if SRC presents with classical features. However, it can help to define prognosis and guide treatment in atypical forms. The prognosis of SRC has dramatically improved with the introduction of angiotensin-converting enzyme inhibitors (ACEi). However, 5 years survival in SSc patients who develop the full picture of SRC remains low (65%). SRC is often triggered by nephrotoxic drugs and/or intravascular volume depletion. The treatment of SRC relies on aggressive control of blood pressure with ACEi, if needed in combination with other types of antihypertensive drugs. Dialysis is frequently indicated, but can be stopped in approximately half of patients, mainly in those for whom a perfect control of blood pressure is obtained. Patients who need dialysis for more than 2 years qualify for renal transplantation. SN - 1559-0267 UR - https://www.unboundmedicine.com/medline/citation/20012923/Scleroderma_renal_crisis:_a_rare_but_severe_complication_of_systemic_sclerosis_ L2 - https://doi.org/10.1007/s12016-009-8191-5 DB - PRIME DP - Unbound Medicine ER -