Renal complications and scleroderma renal crisis

Rheumatology (Oxford). 2009 Jun;48 Suppl 3:iii32-5. doi: 10.1093/rheumatology/ken483.


Scleroderma renal crisis (SRC) occurs in 5-10% of SSc patients, who may present with an abrupt onset of hypertension, acute renal failure, headaches, fevers, malaise, hypertensive retinopathy, encephalopathy and pulmonary oedema. Patients at greatest risk of developing SRC are those with diffuse cutaneous or rapidly progressive forms of SSc, and treatment with a recently commenced high dose of corticosteroid. Laboratory tests may demonstrate hypercreatinaemia, microangiopathic haemolytic anaemia (MAHA), thrombocytopaenia and hyperreninaemia. Renal crisis is also linked to a positive ANA speckled pattern, antibodies to RNA polymerase I and II, and an absence of anti-centromere antibodies. Early, aggressive treatment with angiotensin-converting enzyme inhibitors has improved prognosis in SRC, although 40% of the patients may require dialysis, and mortality at 5 yrs is 30-40%. Median time to recovery is 1 yr, and typically occurs within 3 yrs. Prognosis is worse for males, but may not be related to corticosteroid use, presence of MAHA or severity of renal pathology. Modification of endothelin over-activity, which is implicated in the pathogenesis of SRC, may offer a future therapeutic approach.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Acute Kidney Injury / etiology*
  • Acute Kidney Injury / physiopathology
  • Angiotensin-Converting Enzyme Inhibitors / therapeutic use
  • Autoantibodies / blood
  • Calcium Channel Blockers / therapeutic use
  • Female
  • Glucocorticoids / adverse effects
  • Glucocorticoids / therapeutic use
  • Hormone Replacement Therapy / adverse effects
  • Humans
  • Hypertension, Renal / drug therapy
  • Hypertension, Renal / etiology*
  • Hypertension, Renal / physiopathology
  • Kidney / physiopathology
  • Middle Aged
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / drug therapy
  • Scleroderma, Systemic / physiopathology


  • Angiotensin-Converting Enzyme Inhibitors
  • Autoantibodies
  • Calcium Channel Blockers
  • Glucocorticoids