Tags

Type your tag names separated by a space and hit enter

Diagnosis, management and prevention of scleroderma renal disease.
Curr Opin Rheumatol. 2008 Nov; 20(6):692-6.CO

Abstract

PURPOSE OF REVIEW

Renal complications are important in scleroderma (systemic sclerosis) and include scleroderma renal crisis. This is a medical emergency that requires careful management. Recent cohort studies have highlighted key aspects of management and outcome and these are reviewed.

RECENT FINDINGS

Two recent articles have reported the course of scleroderma renal crisis, and examined risk factors, clinical outcomes and prognostic markers in large contemporary scleroderma cohorts. Diffuse skin disease, early rapidly progressing skin disease, anti-RNA polymerase antibodies and genetic factors are all nonmodifiable risk factors. Use of corticosteroids is associated with scleroderma renal crisis. Over half of cases of scleroderma renal crisis require dialysis; just under half of these will be able to discontinue dialysis over the following 2 years. Despite clear improvement in 12-month survival, which is a testament to the efficacy of angiotensin-converting enzyme inhibitors in renal crisis, long-term mortality remains significant, especially with ongoing dialysis.

SUMMARY

Renal involvement remains a major complication of scleroderma. Long-term outcome after renal crisis remains poor despite the use of angiotensin-converting enzyme inhibitors. There is no evidence at present to support the use of angiotensin-converting enzyme inhibitors prophylactically. The mechanisms and significance of chronic renal impairment in scleroderma need to be better defined.

Authors+Show Affiliations

Centre for Rheumatology, Royal Free Hospital, London, UK.No affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

18946330

Citation

Penn, Henry, and Christopher P. Denton. "Diagnosis, Management and Prevention of Scleroderma Renal Disease." Current Opinion in Rheumatology, vol. 20, no. 6, 2008, pp. 692-6.
Penn H, Denton CP. Diagnosis, management and prevention of scleroderma renal disease. Curr Opin Rheumatol. 2008;20(6):692-6.
Penn, H., & Denton, C. P. (2008). Diagnosis, management and prevention of scleroderma renal disease. Current Opinion in Rheumatology, 20(6), 692-6. https://doi.org/10.1097/BOR.0b013e3283108df7
Penn H, Denton CP. Diagnosis, Management and Prevention of Scleroderma Renal Disease. Curr Opin Rheumatol. 2008;20(6):692-6. PubMed PMID: 18946330.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Diagnosis, management and prevention of scleroderma renal disease. AU - Penn,Henry, AU - Denton,Christopher P, PY - 2008/10/24/pubmed PY - 2009/1/16/medline PY - 2008/10/24/entrez SP - 692 EP - 6 JF - Current opinion in rheumatology JO - Curr Opin Rheumatol VL - 20 IS - 6 N2 - PURPOSE OF REVIEW: Renal complications are important in scleroderma (systemic sclerosis) and include scleroderma renal crisis. This is a medical emergency that requires careful management. Recent cohort studies have highlighted key aspects of management and outcome and these are reviewed. RECENT FINDINGS: Two recent articles have reported the course of scleroderma renal crisis, and examined risk factors, clinical outcomes and prognostic markers in large contemporary scleroderma cohorts. Diffuse skin disease, early rapidly progressing skin disease, anti-RNA polymerase antibodies and genetic factors are all nonmodifiable risk factors. Use of corticosteroids is associated with scleroderma renal crisis. Over half of cases of scleroderma renal crisis require dialysis; just under half of these will be able to discontinue dialysis over the following 2 years. Despite clear improvement in 12-month survival, which is a testament to the efficacy of angiotensin-converting enzyme inhibitors in renal crisis, long-term mortality remains significant, especially with ongoing dialysis. SUMMARY: Renal involvement remains a major complication of scleroderma. Long-term outcome after renal crisis remains poor despite the use of angiotensin-converting enzyme inhibitors. There is no evidence at present to support the use of angiotensin-converting enzyme inhibitors prophylactically. The mechanisms and significance of chronic renal impairment in scleroderma need to be better defined. SN - 1531-6963 UR - https://www.unboundmedicine.com/medline/citation/18946330/Diagnosis_management_and_prevention_of_scleroderma_renal_disease_ L2 - http://dx.doi.org/10.1097/BOR.0b013e3283108df7 DB - PRIME DP - Unbound Medicine ER -