Nephrogenic systemic fibrosis: concepts and perspectives

Author:

Igreja Ana Carolina de Souza Machado1,Mesquita Kleyton de Carvalho2,Cowper Shawn Edwin3,Costa Izelda Maria Carvalho4

Affiliation:

1. UnB, Brazil

2. Universidade de Brasília; State Health Department, Brazil

3. Yale University, USA

4. Universidade de Brasília

Abstract

Nephrogenic systemic fibrosis is a chronic, progressive condition that develops in some patients with renal impairment after exposure to gadolinium-based contrast agents used in magnetic resonance imaging. Thickening of the skin is typical, usually affecting the extremities. Visceral organs can also be affected. The diagnosis of the disease requires careful clinicopathological correlation. Treatment aims at restoring renal function, which is associated with delayed progression and, eventually, remission of skin changes. Reduction and prevention of nephrogenic systemic fibrosis cases are based on limiting the use of gadolinium-based contrast agents in patients with kidney disorders (especially in patients with advanced renal failure at stages 4 and 5), and restricting their use to situations in which they are essential to diagnosis/follow-up. Other than limiting exposure to gadolinium based contrast agents, no effective preventive methods have been reported. Due to increased awareness about the disease among radiologists and nephrologists, the incidence of nephrogenic systemic fibrosis is declining.

Publisher

FapUNIFESP (SciELO)

Subject

Dermatology

Reference54 articles.

1. Scleromyxoedema-like cutaneous diseases in renal-dialysis patients;Cowper SE;Lancet,2000

2. Nephrogenic Systemic Fibrosis: Current Concepts;Basak P;Indian J Dermatol,2011

3. Dermatologic conditions seen in end-stage renal disease;Cordova KB;Semin Dial,2009

4. Nephrogenic systemic fibrosis: a severe complication of use to gadolinium in patients with kidney failure;Mundim JS;Rev Assoc Med Bras,2009

5. Large sample of nephrogenic systemic fibrosis cases from a single institution;Lee CU;Arch Dermatol,2009

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