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scleroderma renal crisis

Etiology: 1) almost exclusively with early cutaneous scleroderma 2) risk factors: a) glucocorticoid use b) pericardial effusion Epidemiology: - 10-15% of patients with systemic sclerosis - more frequent in patients with scleroderma than limited cutaneous sclerosis - 80% female Pathology: - intimal proliferation & luminal thrombosis in the afferent renal arterioles - thrombotic microangiopathy with glomerular ischemia - hyperreninemia Clinical manifestations: 1) hypertension often severe - some patients may be normotensive (10%) [1] 2) renal failure 3) microangiopathic hemolytic anemia 4) variable features - pulmonary edema - hypertensive encephlopathy [1] Laboratory: - complete blood count may show anemia, thrombocytopenia - peripheral blood smear may show microangiopathic hemolytic anemia - renal function tests consistent with acute renal failure - plasma renin is high [1] - RNA polymerase 3 Ab in serum Management: 1) a medical emergency 2) hospitalization for aggressive blood pressure control 3) ACE inhibitors are standard therapy even in the face of renal failure (including dialysis) regardless of blood pressure [1] - captopril is the preferred ACE inhibitor [1] - most extensively studied - short 1/2 life allows rapid titration [1] 4) hemodialysis as needed - 65% of patients require hemodialysis [3] - renal function may improve months after initiation of dialysis [1] 5) prognosis - 5 year mortality 40%, higher in men

General

scleroderma (diffuse cutaneous systemic sclerosis) acute renal failure (ARF)

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 15,16,17,18. American College of Physicians, Philadelphia 2009,2012,2015,2018
  2. Denton CP. Renal manifestations of systemic sclerosis--clinical features and outcome assessment. Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v54-6 PMID: 18784147
  3. Penn H, Howie AJ, Kingdon EJ et al Scleroderma renal crisis: patient characteristics and long- term outcomes. QJM. 2007 Aug;100(8):485-94. Epub 2007 Jun 29 PMID: 17601770
  4. Hudson M, Baron M, Lo E, Weinfeld J, Furst DE, Khanna D. An International, Web-Based, Prospective Cohort Study to Determine Whether the Use of ACE Inhibitors prior to the Onset of Scleroderma Renal Crisis Is Associated with Worse Outcomes-Methodology and Preliminary Results. Int J Rheumatol. 2010;2010. pii: 347402. PMID: 20936135
  5. Woodworth TG, Suliman YA, Li W, Furst DE, Clements P. Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol. 2016 Nov;12(11):678-691. Review. PMID: 27641135