Fibromuscular dysplasia mimicking Crohn's disease over a period of 23 years.

ABSTRACT

Rare diseases with similar clinical presentation as more frequent gastrointestinal disorders might be challenging in the diagnostic and therapeutic management. In this case we report on a 47-year-old woman who was thought to suffer from Crohn's disease. Symptoms, macroscopic and histological aspects of the gastrointestinal tract, treatment response and clinical course had encouraged the wrong diagnosis over a period of 23 years. After the patient died in the context of a sudden clinical deterioration, fibromuscular dysplasia of the aorta was finally unmasked by post-mortem examination as underlying cause of all symptoms attributed to Crohn's disease. Re-evaluation of former diagnostic procedures revealed subtle aspects of fibromuscular dysplasia, even in biopsy samples from 23 years ago. This first case report of fibromuscular dysplasia of the aorta documents a rare pitfall in the diagnostic workup of a frequent clinical presentation in gastroenterology.