title: Recent Developments in the Classification, Evaluation, Pathophysiology, and Management of Scleroderma Renal Crisis.
abstract: Scleroderma renal crisis (SRC) is an uncommon complication of systemic sclerosis. Despite the advent of angiotensin-converting inhibitor therapy, SRC remains a life-threatening complication. Recent studies have contributed to a better understanding of SRC, but much remains unknown regarding its pathophysiology, risk factors, and optimal management. Genetic studies provide evidence that immune dysregulation might be a contributing factor, providing hope that further research in this direction might illuminate pathogenesis and provide novel predictors for this complication.
authors.affiliation: Department of Medicine, Division of Nephrology, Northwestern University, Feinberg School of Medicine, 710 North Fairbanks Court-Suite 4500, Chicago, IL, 60611, USA. c-ghossein@northwestern.edu.
authors.forename: Cybele
authors.initials: C
authors.lastname: Ghossein
authors.affiliation: Department of Medicine, Northwestern University, Feinberg School of Medicine, 240 E Huron Street McGaw Pavilion Rheumatology M300, Chicago, IL, 60611, USA. j-varga@northwestern.edu.|Department of Medicine, Division of Rheumatology, Northwestern University, Feinberg School of Medicine, 240 E Huron Street McGaw Pavilion Rheumatology M300, Chicago, IL, 60611, USA. j-varga@northwestern.edu.
authors.forename: John
authors.initials: J
authors.lastname: Varga
authors.affiliation: Department of Medicine, Massachusetts General Hospital, Harvard Medical School, 50 Staniford Street, Suite 503B, Boston, MA, 02114, USA. AFENVES@mgh.harvard.edu.
authors.forename: Andrew Z
authors.initials: AZ
authors.lastname: Fenves
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D006801:Humans; D016030:Kidney Transplantation; D011379:Prognosis; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors
keywords: Angiotensin-converting enzyme inhibitor (ACEI); Diffuse cutaneous disease (dcSSc); Immune dysregulation; Malignant hypertension; Renal biopsy; Scleroderma renal crisis (SRC); Systemic sclerosis; Thrombotic microangiopathy