Nephrogenic systemic fibrosis

Previously called nephrogenic fibrosing dermopathy.

A rare disorder primarily involving the skin with the fibrosis in patients with renal failure, especially those on long-term hemodialysis.

Fibrosis may be systemic and extend to muscle, heart, and lung.

Always occurs in patients with renal insufficiency who have had imaging studies.

In patients with severely compromised kidney function, there is a progressive accumulation of gadolinium in skin tissues over time, even in the absence of additional gadolinium based contrast agents, suggesting mobilization from tissue reservoirs such as bone.

Dechelation, the process of disassociation of the gadolinium from the organic ligand is thought to play a role in the disease.

Patients should be screened for acute and chronic kidney disease prior to contrast enhanced MRI.

May be associated with gadolinium contrast agents utilized in MRI enhancements.

Gadolinium can be found in tissue of nephrogenic systemic fibrosis.

Resembles scleroderma and eosinophilic fasciitis clinically and scleromyxedema histopathologically.

May develop large areas of indurated skin with fibrotic nodules and plaques.

Flexion contractures may limitation of range of motion.

Some patients have never undergone dialysis and others have received only peritoneal dialysis.

Histopathologically has a proliferation of dermal fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers, and mucin deposition.

More than 90% of proven nephrogenic systemic fibrosis cases are related to gadolinium.

Only seen in patients with severe renal insufficiency,

Resembles eosinophilia-myalgia syndrome.

Only tiny concentrations of gadolinium are needed to stimulate hyaluronan synthesis by fibroblasts.

Anticardiolipin or antiphospholipid antibodies are common in patients with nephrogenic systemic fibrosis.

The incidence of nephrogenic systemic fibrosis is 4.3 cases per 1000 patient-years.

Each radiologic study using gadolinium presents a 2.4% risk for developing nephrogenic systemic fibrosis.

Associated with increased morbidity and mortality.

Many patients become dependent on a wheelchair because of contractures within weeks to months of disease onset.

Patients may report pruritus and/or causalgia.

No racial or sexual prediliction.

Reported in all age groups.

Not correlated with the duration of renal failure.

May be associated with abnormal calcium metabolism and calciphylaxis.

Some patients have known concurrent liver disease, hepatitis C and/or neoplasia.

May have associated hypercoaguable state.

Manifests with induration, thickening, and hardening of the skin with hyperpigmentation.

Peau d’orange appearance may be present.

The skin is often shiny and hard.

Extremities are the most common areas of involvement, followed by the trunk.

Debilitating and sometimes fatal.

Differential diagnosis includes: Calciphylaxis, cellulitis, morphea, dermatofibrosarcoma protuberans, eosinophilia-myalgia syndrome, systemic sclerosis, granuloma annulare, and amyloidosis.

Skin biopsy should be performed for diagnostic purposes.

Rarely partial or complete spontaneous resolution have been reported.

Extracorporeal photopheresis is the best treatment modality.

Cases have reportedly responded to high-dose intravenous immunoglobulins, UV light treatment, alpha interferon, plasmapheresis,intravenous thiosulfate and corticosteroids.

Kidney transplantation may cause resolution of lesions.

No therapy has been effective and renal transplantation may be the best option.

Leave a Reply

Your email address will not be published. Required fields are marked *