title: Renal disease in scleroderma:  an update on evaluation, risk stratification, pathogenesis and management.
abstract: OBJECTIVE
  Renal disease remains an important cause of morbidity and mortality in scleroderma. The spectrum of renal complications in systemic sclerosis includes scleroderma renal crisis (SRC), normotensive renal crisis, antineutrophil cytoplasmic antibodies-associated glomerulonephritis, penacillamine-associated renal disease, and reduced renal functional reserves manifested by proteinuria, microalbuminuria, or isolated reduction in glomerular filtration rate. The purpose of this review is to provide a concise and up-to-date review of the evaluation, risk stratification, pathogenesis, and management of scleroderma-associated renal disease.
  
  
  RESULTS
  Although SRC survival has significantly improved, mortality of this complication remains high outside of specialized centers. Recent data demonstrate strong associations between anti-RNA polymerase III antibodies and SRC. Subclinical renal impairment affects approximately 50% of scleroderma patients and may be associated with other vascular manifestations. Subclinical renal involvement rarely progresses to end-stage renal failure; however, recent studies suggest it may predict mortality in patients with other vasculopathic manifestations.
  
  
  CONCLUSIONS
  Testing for anti-RNA polymerase III antibodies should be incorporated into clinical care to identify patients at high risk for SRC. Recommendations from European League Against Rheumatism (EULAR), EULAR Scleroderma Trials and Research, and the Scleroderma Clinical Trials Consortium confirm angiotensin-converting enzyme inhibitors as first-line therapy for SRC, and give recommendations for second-line agents.
authors.affiliation: Division of Rheumatology, Immunology and Allergy, MedStar Georgetown University Hospital, Washington, DC 20007, USA. vks4@gunet.georgetown.edu
authors.forename: Victoria K
authors.initials: VK
authors.lastname: Shanmugam
authors.forename: Virginia D
authors.initials: VD
authors.lastname: Steen
mesh_terms: D058186:Acute Kidney Injury / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000097:blood; D006801:Humans; D016030:Kidney Transplantation; D011379:Prognosis; D012320:RNA Polymerase III / Q000276:immunology; D051437:Renal Insufficiency / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D018570:Risk Assessment; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D015996:Survival Rate
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D000974:Antibodies, Antinuclear; D012320:RNA Polymerase III