Nephrogenic systemic fibrosis: early recognition and treatment.

Nephrogenic systemic fibrosis (NSF) is a progressive, debilitating, and emotionally distressing disease that can affect patients with renal dysfunction. Prevention, early recognition and early treatment are essential to limiting its impact. The most significant risk factors for developing NSF are chronic or significant acute kidney disease (usually necessitating dialysis) and the administration of gadolinium-containing contrast agents (GCCA). Early symptoms include swelling, redness, pruritus, and pain in the limbs, sometimes with muscle weakness. Early signs are edema, erythema, and occasionally palpable warmth of the involved extremities; there may be florid scleral telangiectasia resembling conjunctivitis. We must redouble our efforts to avoid the administration of GCCA to patients with renal insufficiency. The most effective treatment for NSF to date is maximization of renal function via medical therapy or transplantation. There are data to support a beneficial effect from extracorporeal photopheresis, and all patients can gain from physical therapy.