Scleroderma pp 361-371 | Cite as

Scleroderma Renal Crisis

  • Christopher P. Denton
  • Virginia Steen


Systemic sclerosis (SSc) is a multisystem autoimmune rheumatic disease that results in substantial morbidity and significant mortality. Indeed, it is the autoimmune rheumatic disease with the highest case-specific mortality, and this occurs as a result of organ-based complications. Historically, renal manifestations were a major cause of death, but recent series show that most patients dying from SSc do so as a result of cardiopulmonary complications including, pulmonary hypertension, lung fibrosis, and myocardial involvement. (Nihtyanova SI, Tang EC, Coghlan JG, Wells AU, Black CM, Denton CP. Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM. 2010;103(2):109–15.) In many cases, there is co-occurrence of these different manifestations, and this may account for the particular severity of SSc. Although there are a range of ways in which the kidneys can be affected in SSc, the most significant renal manifestation is the scleroderma renal crisis (SRC), and this was historically a major cause of scleroderma-associated death.


ACEi anti-RNA polymerase III glomerular hypoperfusion glomerulonephritis microangiopathic renin schistocytes 


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© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Department of Inflammation, Division of Medicine, Centre for Rheumatology and Connective Tissue DiseasesUCL Medical SchoolLondonUK
  2. 2.Department of Medicine, Rheumatology, Clinical Immunology and AllergyGeorgetown University HospitalWashingtonUSA

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