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Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls.
Rheumatology (Oxford). 2012 Mar; 51(3):460-7.R

Abstract

OBJECTIVE

Scleroderma renal crisis (SRC) is a severe manifestation of SSc, whose prognosis remains severe, despite treatment with angiotensin-converting-enzyme inhibitor and dialysis. This study was undertaken to describe SRC characteristics, prognosis and outcome, and evaluate the responsibility of CSs in its occurrence.

METHODS

Analysis concerned 91 SSc patients with SRC who were compared with 427 non-SRC-SSc patients taken as controls.

RESULTS

Among the 91 SRC patients, 71 (78.0%) had high blood pressure, 53 (58.2%) hypertensive encephalopathy and 51 (56.0%) thrombotic microangiopathy; 64 (70.3%) had received CSs before or concomitantly with SRC vs 156 (36.5%) non-SRC-SSc patients (P < 0.001). Treated SRC patients also received more prednisone 29.3 (28.4) vs 3.6 (9.9) mg than controls (P < 0.001). SRC clinical outcomes were poor: 49 (53.8%) patients required dialysis, which was definitive for 38. Thirty-seven (40.7%) SRC patients died vs 10.8% of the controls (P < 0.001). Death was most frequent among dialysed patients who never recovered renal function (22 vs 2) and 13 never-dialysed SRC patients died.

CONCLUSIONS

Although SRC prognosis has improved markedly, SRC remains a severe manifestation of SSc, despite treatment with angiotensin-converting enzyme inhibitor and dialysis. CSs contributed significantly to SRC occurrence.

Authors+Show Affiliations

Department of Internal Medicine, Hôpital Cochin, Paris Cedex 14, France. loic.guillevin@cch.aphp.frNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Multicenter Study
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

22087012

Citation

Guillevin, Loïc, et al. "Scleroderma Renal Crisis: a Retrospective Multicentre Study On 91 Patients and 427 Controls." Rheumatology (Oxford, England), vol. 51, no. 3, 2012, pp. 460-7.
Guillevin L, Bérezné A, Seror R, et al. Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls. Rheumatology (Oxford). 2012;51(3):460-7.
Guillevin, L., Bérezné, A., Seror, R., Teixeira, L., Pourrat, J., Mahr, A., Hachulla, E., Agard, C., Cabane, J., Vanhille, P., Harle, J. R., Deleveaux, I., & Mouthon, L. (2012). Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls. Rheumatology (Oxford, England), 51(3), 460-7. https://doi.org/10.1093/rheumatology/ker271
Guillevin L, et al. Scleroderma Renal Crisis: a Retrospective Multicentre Study On 91 Patients and 427 Controls. Rheumatology (Oxford). 2012;51(3):460-7. PubMed PMID: 22087012.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls. AU - Guillevin,Loïc, AU - Bérezné,Alice, AU - Seror,Raphaèle, AU - Teixeira,Luis, AU - Pourrat,Jacques, AU - Mahr,Alfred, AU - Hachulla,Eric, AU - Agard,Christian, AU - Cabane,Jean, AU - Vanhille,Philippe, AU - Harle,Jean-Robert, AU - Deleveaux,Isabelle, AU - Mouthon,Luc, Y1 - 2011/11/15/ PY - 2011/11/17/entrez PY - 2011/11/17/pubmed PY - 2012/4/20/medline SP - 460 EP - 7 JF - Rheumatology (Oxford, England) JO - Rheumatology (Oxford) VL - 51 IS - 3 N2 - OBJECTIVE: Scleroderma renal crisis (SRC) is a severe manifestation of SSc, whose prognosis remains severe, despite treatment with angiotensin-converting-enzyme inhibitor and dialysis. This study was undertaken to describe SRC characteristics, prognosis and outcome, and evaluate the responsibility of CSs in its occurrence. METHODS: Analysis concerned 91 SSc patients with SRC who were compared with 427 non-SRC-SSc patients taken as controls. RESULTS: Among the 91 SRC patients, 71 (78.0%) had high blood pressure, 53 (58.2%) hypertensive encephalopathy and 51 (56.0%) thrombotic microangiopathy; 64 (70.3%) had received CSs before or concomitantly with SRC vs 156 (36.5%) non-SRC-SSc patients (P < 0.001). Treated SRC patients also received more prednisone 29.3 (28.4) vs 3.6 (9.9) mg than controls (P < 0.001). SRC clinical outcomes were poor: 49 (53.8%) patients required dialysis, which was definitive for 38. Thirty-seven (40.7%) SRC patients died vs 10.8% of the controls (P < 0.001). Death was most frequent among dialysed patients who never recovered renal function (22 vs 2) and 13 never-dialysed SRC patients died. CONCLUSIONS: Although SRC prognosis has improved markedly, SRC remains a severe manifestation of SSc, despite treatment with angiotensin-converting enzyme inhibitor and dialysis. CSs contributed significantly to SRC occurrence. SN - 1462-0332 UR - https://www.unboundmedicine.com/medline/citation/22087012/Scleroderma_renal_crisis:_a_retrospective_multicentre_study_on_91_patients_and_427_controls_ L2 - https://academic.oup.com/rheumatology/article-lookup/doi/10.1093/rheumatology/ker271 DB - PRIME DP - Unbound Medicine ER -