Scleroderma renal crisis.

Abstract

There are important knowledge gaps concerning scleroderma renal crisis (SRC), in large part because its rarity has hampered research. Many studies to date share limitations, in particular small samples, prevalent cases, and retrospective study designs. This review features some of the most recent studies that attempt to shed new insights into SRC while trying to overcome those limitations. The most important recent progress in the understanding of the pathophysiology of SRC includes identification of novel genetic and serological biomarkers. Outcomes of SRC remain poor and there are also ongoing efforts to identify novel therapeutic strategies, in particular targeting the endothelin-1 pathway. Meaningful improvement in outcomes of SRC will be predicated on greater understanding of the underlying mechanisms of disease and identification of novel therapeutic and preventive strategies. Some efforts are ongoing but ultimately, international cooperation will be necessary to achieve this for a rare complication of a rare disease.