Nephrogenic systemic fibrosis, initially called nephrogenic fibrosing dermopathy, has been strongly linked to exposure to gadolinium-based contrast media used in magnetic resonance imaging in patients with renal insufficiency. This review discusses recent advances in our understanding of the pathophysiology and clinical approach to patients with chronic kidney disease who require diagnostic ima...

Nephrogenic systemic fibrosis (NSF) is a recently identified idiopathic cutaneous fibrosing disorder that occurs in the setting of renal failure. The disease initially called nephrogenic fibrosing dermopathy is closely linked to exposure to gadolinium-based contrast media used during magnetic resonance imaging in patients with renal insufficiency. Although little is known about the pathogenesis...

Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is a rare complication of exposure to gadolinium-based contrast agents in patients who have significantly decreased renal function. Manifestations include fibrosis of the skin and other tissues. Effective therapies are lacking. Photopheresis has been tried with variable rates of improvement, and small num...

A 47-year-old woman presented with a 2-month history of progressive tightening of the skin on her legs. The tightening of her skin had begun 2 weeks after she was hospitalized for acute renal failure precipitated by therapy with nonsteroidal anti-inflammatory drugs. She had no history of renal disease or medical conditions that would predispose her to the organ failure. During her hospitalizati...

Abbreviations: BBB: Blood Brain Barrier; Ca: Calcium; CT: Computed Tomography; EM: Electro-Magnetic; EMA: European Medicines Agency; FDA: (U.S.) Food And Drug Administration; Fmri: Functional Magnetic Resonance Imaging; Gd: Gadolinium; GI: Gastro-Intestinal; IV: Intra-Venous; Mn: Manganese; MEMRI: Manganese-Enhanced MRI; Mn-DPDP: Chelated Manganese Nanoparticles; MRA: Magnetic Resonance Angiogr...

Nephrogenic systemic fibrosis is a disabling progressive condition that is being reported with increased frequency in patients with kidney disease. Treatment is extremely limited and largely supportive. We report a case of severe nephrogenic systemic fibrosis in a dialysis patient exposed to multiple doses of gadolinium who improved clinically and histologically with treatment with imatinib.