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Scleroderma renal crisis: patient characteristics and long-term outcomes.
QJM. 2007 Aug; 100(8):485-94.QJM

Abstract

BACKGROUND

Scleroderma renal crisis (SRC) is an important complication of systemic sclerosis, causing acute renal failure, and usually hypertension.

AIMS

To review the clinical and pathological features of SRC, and correlate them with renal outcomes and mortality.

DESIGN

Retrospective case series.

METHODS

We identified 110 cases of SRC managed at a single centre between 1990 and 2005.

RESULTS

SRC occurred in 5% of scleroderma cases under follow-up. Cases were predominantly female (81%), with diffuse cutaneous disease (78%). RNA polymerase antibodies were found in 59% of cases tested. Almost all (108/110) received treatment with ACE inhibitors (ACEIs). Dialysis was not required in 36%, was required temporarily (for up to 3 years) in 23%, was required permanently in 41%. Patients not on dialysis showed improvement in estimated glomerular filtration rate after SRC (mean change +23 ml/min over 3 years). Poor renal outcome was associated with lower blood pressure at presentation, and with higher age in those requiring dialysis. Steroid use, microangiopathic haemolytic anaemia, and antibody profile were not related to renal outcome. In the 58 renal biopsies available for clinical correlation, acute changes of mucoid intimal thickening in arteries and fibrinoid necrosis in arterioles were associated with a poorer renal outcome. Mortality was high (59% survival at 5 years), and was higher in men.

DISCUSSION

Despite the efficacy of ACEIs in managing SRC, the poor long-term outcome warrants evaluation for additional treatments for this devastating complication of systemic sclerosis.

Authors+Show Affiliations

Department of Rheumatology, Royal Free Hospital, London, UK. h.penn@medsch.ucl.ac.ukNo 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

Language

eng

PubMed ID

17601770

Citation

Penn, H, et al. "Scleroderma Renal Crisis: Patient Characteristics and Long-term Outcomes." QJM : Monthly Journal of the Association of Physicians, vol. 100, no. 8, 2007, pp. 485-94.
Penn H, Howie AJ, Kingdon EJ, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM. 2007;100(8):485-94.
Penn, H., Howie, A. J., Kingdon, E. J., Bunn, C. C., Stratton, R. J., Black, C. M., Burns, A., & Denton, C. P. (2007). Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM : Monthly Journal of the Association of Physicians, 100(8), 485-94.
Penn H, et al. Scleroderma Renal Crisis: Patient Characteristics and Long-term Outcomes. QJM. 2007;100(8):485-94. PubMed PMID: 17601770.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Scleroderma renal crisis: patient characteristics and long-term outcomes. AU - Penn,H, AU - Howie,A J, AU - Kingdon,E J, AU - Bunn,C C, AU - Stratton,R J, AU - Black,C M, AU - Burns,A, AU - Denton,C P, Y1 - 2007/06/29/ PY - 2007/7/3/pubmed PY - 2008/1/26/medline PY - 2007/7/3/entrez SP - 485 EP - 94 JF - QJM : monthly journal of the Association of Physicians JO - QJM VL - 100 IS - 8 N2 - BACKGROUND: Scleroderma renal crisis (SRC) is an important complication of systemic sclerosis, causing acute renal failure, and usually hypertension. AIMS: To review the clinical and pathological features of SRC, and correlate them with renal outcomes and mortality. DESIGN: Retrospective case series. METHODS: We identified 110 cases of SRC managed at a single centre between 1990 and 2005. RESULTS: SRC occurred in 5% of scleroderma cases under follow-up. Cases were predominantly female (81%), with diffuse cutaneous disease (78%). RNA polymerase antibodies were found in 59% of cases tested. Almost all (108/110) received treatment with ACE inhibitors (ACEIs). Dialysis was not required in 36%, was required temporarily (for up to 3 years) in 23%, was required permanently in 41%. Patients not on dialysis showed improvement in estimated glomerular filtration rate after SRC (mean change +23 ml/min over 3 years). Poor renal outcome was associated with lower blood pressure at presentation, and with higher age in those requiring dialysis. Steroid use, microangiopathic haemolytic anaemia, and antibody profile were not related to renal outcome. In the 58 renal biopsies available for clinical correlation, acute changes of mucoid intimal thickening in arteries and fibrinoid necrosis in arterioles were associated with a poorer renal outcome. Mortality was high (59% survival at 5 years), and was higher in men. DISCUSSION: Despite the efficacy of ACEIs in managing SRC, the poor long-term outcome warrants evaluation for additional treatments for this devastating complication of systemic sclerosis. SN - 1460-2725 UR - https://www.unboundmedicine.com/medline/citation/17601770/Scleroderma_renal_crisis:_patient_characteristics_and_long_term_outcomes_ L2 - https://academic.oup.com/qjmed/article-lookup/doi/10.1093/qjmed/hcm052 DB - PRIME DP - Unbound Medicine ER -