Marie Hudson1. 1. Division of Rheumatology and Lady Davis Institute for Medical Research, Jewish General Hospital, and Faculty of Medicine, McGill University, Montréal, Québec, Canada.
Abstract
PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: 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.
PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: 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.
Authors: Lorinda Chung; Cathie Spino; Richard McLain; Sindhu R Johnson; Christopher P Denton; Jerry A Molitor; Virginia D Steen; Robert Lafyatis; Robert W Simms; Suzanne Kafaja; Tracy M Frech; Vivien Hsu; Robyn T Domsic; Janet E Pope; Jessica K Gordon; Maureen D Mayes; Nora Sandorfi; Faye N Hant; Elana J Bernstein; Soumya Chatterjee; Flavia V Castelino; Ali Ajam; Yannick Allanore; Marco Matucci-Cerinic; Michael L Whitfield; Oliver Distler; Ora Singer; Amber Young; Vivek Nagaraja; David A Fox; Daniel E Furst; Dinesh Khanna Journal: Lancet Rheumatol Date: 2020-10-19