Nephrogenic systemic fibrosis with multiple calcification and osseous metaplasia

Area of Science:

• Nephrology
• Dermatology
• Radiology

Background:

• Nephrogenic Systemic Fibrosis (NSF) is a rare, debilitating condition linked to gadolinium-based contrast agents (GBCAs) in patients with renal impairment.
• Chronic renal insufficiency and haemodialysis are significant risk factors for developing NSF.
• Magnetic Resonance Angiography (MRA) is a diagnostic tool that utilizes GBCAs, necessitating careful patient selection.

Observation:

• A 50-year-old Japanese male with chronic renal insufficiency on haemodialysis presented with symptoms of NSF.
• Symptoms included progressive swelling, induration, pain, and limited range of motion in extremities, starting one month post-MRA with gadodiamide.
• Physical examination revealed characteristic skin sclerosis with a glossy, brownish appearance.

Findings:

• Histological examination showed increased dermal and subcutaneous collagen, high cellularity, thickened fascia, and subfascial osseous metaplasia.
• Whole-body computed tomography confirmed widespread fascial calcification.
• Intravenous sodium thiosulphate treatment yielded no clinical improvement.

Implications:

• This case highlights the persistent risk of NSF even years after GBCA exposure in susceptible individuals.
• The findings underscore the importance of risk assessment and alternative imaging strategies in patients with renal insufficiency.
• Further research into effective treatments for established NSF is warranted, given the limitations of current therapeutic options.