Diagnosis, management and prevention of scleroderma renal disease

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.

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.

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.