Snapshot A 56-year-old man presents to his primary care physician with muscle weakness and joint contractures in his hands. He reports to having developed a painful rash that feels hard and states he has a general “tightness” in his body. He has a past medical history of type II diabetes mellitus and end-stage renal disease treated with hemodialysis. About 1 month prior to presentation, the patient had a transient ischemic attack and underwent an MRI with contrast of the brain. On physical exam, he has bilateral symmetric indurated papules and plaques on his forearms and shins, his hands have contractures, and his range of motion is limited in the involved joints. Laboratory evaluation reveals an elevated erythrocyte sedimentation rate, C-reactive protein, and ferritin. Introduction Clinical definition rare systemic fibrosing disorder in patients with renal failure that is associated with gadolinium-containing contrast agent primarily characterized by thickening of skin fibrosis of dermis, but can also affect muscle, fascia, lungs, and heart Epidemiology Incidence rare Demographics occurs only in patients with renal failure Risk factors renal failure renal transplant chronic kidney disease exposure to gadolinium-containing agents Etiology Gadolinium an active component in contrast agents used in MRI studies Pathogenesis mechanism is unclear gadolinium may dissociate from its chelating molecule and increase the exposure in patients with renal failure gadolinium deposition in tissue may cause a tissue injury reaction this may activate transforming growth factor TGF-β1 pathway gadolinium may also directly increase the number of circulating fibrocytes by stimulating the bone marrow increased number of fibrocytes can produce collagen and cause tissue fibrosis Presentation Symptoms onset occurs 2-4 weeks after exposure to gadolinium but can occur up to years after exposure skin lesions start at extremities and move proximally to thighs and forearms common distribution ankles to below the knees skin between wrists and upper arms head is spared pruritic or painful lung involvement shortness of breath Physical exam skin lesions symmetric and bilateral indurated papules and plaques subcutaneous nodules edematous cobblestone or peau d’orange appearance limited range of motion joint contractures sclerodactyly Imaging CT scan with contrast indications evaluation of systemic organ involvement findings fibrosis of fascia and muscles Studies Labs ↑ erythrocyte sedimentation rate ↑ C-reactive protein ↑ ferritin Biopsy reaching subcutaneous tissue proliferation of CD34+ dermal fibrocytes Differential Scleroderma presence of Raynaud phenomenon Treatment No medical intervention proven to be effective The following may have some benefit conservative physical therapy indications for all patients to prevent or reverse immobility from disease non-operative extracorporeal photopheresis indications if disease progresses ultraviolet A phototherapy indications if disease progresses often used in combination with other therapies Complications Respiratory failure from lung involvement Wheelchair dependence due to immobility Prognosis 30% mortality Positive prognostic factors recovery of renal function