scleroderma renal crisis
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Etiology
- almost exclusively with early cutaneous scleroderma
- risk factors:
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
- hypertension often severe
- some patients may be normotensive (10%)[1]
- renal failure
- microangiopathic hemolytic anemia
- 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
- a medical emergency
- hospitalization for aggressive blood pressure control
- ACE inhibitors are standard therapy even in the face of renal failure (including dialysis) regardless of blood pressure[1]
- hemodialysis as needed
- 65% of patients require hemodialysis[3]
- renal function may improve months after initiation of dialysis[1]
- prognosis
- 5 year mortality 40%, higher in men
More general terms
References
- ↑ Jump up to: 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 Medical Knowledge Self Assessment Program (MKSAP) 15,16,17,18. American College of Physicians, Philadelphia 2009,2012,2015,2018
- ↑ Denton CP. Renal manifestations of systemic sclerosis--clinical features and outcome assessment. Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v54-6 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18784147
- ↑ Jump up to: 3.0 3.1 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: https://www.ncbi.nlm.nih.gov/pubmed/17601770
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/20936135
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/27641135