Nephrogenic systemic fibrosis

Nephrogenic systemic fibrosis (NSF), formerly known as nephrogenic fibrosing dermopathy, is a rare acquired fibrosing disorder that occurs almost exclusively in patients with severe renal insufficiency (particularly those on dialysis or with a glomerular filtration rate below 30 mL/min). The condition is strongly associated with exposure to gadolinium-based contrast agents (GBCAs) used in magnetic resonance imaging (MRI). NSF primarily affects the skin but can also involve internal organs including the lungs, heart, liver, and skeletal muscles, making it a truly systemic disease. The hallmark clinical features include progressive hardening and thickening of the skin, typically beginning on the extremities and sometimes extending to the trunk. Patients develop firm, indurated plaques and papules with a woody texture, often accompanied by skin darkening or reddening. Joint contractures may develop as fibrosis progresses, leading to significant disability and reduced mobility. Some patients experience burning, itching, or sharp pain in affected areas. In severe cases, fibrosis of internal organs can lead to life-threatening complications. There is no consistently effective treatment for NSF. Management strategies focus on improving renal function when possible, as restoration of kidney function (including renal transplantation) has been associated with partial or complete resolution of symptoms in some patients. Physical therapy is important to maintain mobility and prevent contractures. Various therapies including extracorporeal photopheresis, plasmapheresis, sodium thiosulfate, and immunosuppressive agents have been tried with variable results. Prevention remains the most critical strategy, and current guidelines strongly recommend avoiding or minimizing the use of high-risk GBCAs in patients with advanced kidney disease.

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