What is nephrogenic systemic fibrosis?
Nephrogenic systemic fibrosis (NSF) is a rare, but devastating, cutaneous fibrosing
dermopathy seen in patients with end-stage kidney disease who have been exposed to the
gadolinium-based contrast agents used in MRI. Onset usually within 2–10 weeks of
exposure. NSF may affect the lungs, heart, liver, and muscles, as well as the skin and
subcutaneous tissues. Complications, such as cardiomyopathy, pulmonary fibrosis,
pulmonary hypertension, and/or diaphragmatic paralysis, may be fatal (mortality rate
30%).
Gadolinium is deposited in affected tissues. Circulating fibrocytes may be involved in the
pathogenesis of fibrosis, and certain factors increase the risk of developing NSF.
More information :
• Symptoms: progressive painful tightening of skin, leading to disabling joint
contractures. Bone pain is common.
• Has the patient been investigated by a contrast-enhanced MRI in previous weeks or
months?
• Explore other causes of skin thickening. For example, symptoms such as itch, Raynaud
phenomenon, or difficulty swallowing suggest systemic sclerosis, rather than NSF.
Symptoms :
• Symmetrical erythematous, oedematous, hyperpigmented patches or plaques most often
on the lower limbs, but may affect the trunk and upper limbs. The face and neck are
spared.
• Papules, nodules, and/or well-demarcated indurated plaques with an irregular outline.
• Woody thickening of the skin and fascia gives a cobblestone or ‘peau d’orange’
appearance.
• Yellow scleral plaques, especially in patients under 45 years old.
• Normal nail folds (unlike systemic sclerosis).
• Flexion contractures of joints in association with skin thickening.
Treatment :
• Take a deep elliptical skin biopsy, looking for fibrosis, dermal fibroblast-like cells
(CD34positive), thickening of collagen, and increased elastic and mucin.
• Consider a muscle biopsy.
• Exclude other causes of skin thickening :
o FBC (no eosinophilia), and anti-Scl-70 and anti-centromere antibodies (absent).
ANA may be positive in NSF and is not a helpful discriminator; paraproteins
absent; thyroid antibodies absent.
• Echocardiogram, chest X-Ray, lung function to exclude systemic disease.
• May improve if renal function improves.