What is the prognosis of nephrogenic systemic fibrosis (NSF)?

Updated: May 22, 2018
  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Spontaneous resolution is described in some reports, typically coincident with improved/resolved renal disease. In most patients, nephrogenic systemic fibrosis is a progressive condition. Many patients report stabilization and marginal improvement after years with the condition. Whether nephrogenic systemic fibrosis is a localized or systemic disease is not yet clear.

Nephrogenic systemic fibrosis appears linked to increased morbidity and mortality. Todd et al [33] found that 24-month mortality rates following examination were 48% and 20% in patients with and those without cutaneous changes of nephrogenic systemic fibrosis, respectively (adjusted hazard ratio, 2.9; 95% confidence interval, 1.4-5.9).

Within weeks of disease onset, many patients become dependent on a wheelchair because of contractures. Several patients have died because of complications from fractures after falls triggered by their mobility problems. Additionally, many patients report maddening pruritus and/or causalgia. Finally, some patients experience flexion contractures if the disorder occurs over a joint.


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