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Diffuse intimal fibromuscular dysplasia with multiorgan failure
J B Stokes1, S M Bonsib, J W McBride
1Department of Internal Medicine, University of Iowa College of Medicine, Iowa City, USA.
Archives of Internal Medicine
|December 9, 1996
Summary
Fibromuscular dysplasia (FMD) can be a widespread, fast-progressing arterial disease. This case highlights FMD
Area of Science:
- Vascular Biology and Medicine
- Pathology and Disease Mechanisms
- Surgical Case Reports
Background:
- Fibromuscular dysplasia (FMD) is a rare, non-atherosclerotic vascular disease.
- Intimal FMD, a subtype, can affect multiple arterial beds.
- The clinical presentation and progression of diffuse intimal FMD are not well-characterized.
Observation:
- A patient presented with acute hypertension, angina, peripheral vascular disease, and renal compromise.
- A large liver cyst was surgically removed, leading to acute renal and liver failure.
- The patient experienced a terminal arrhythmia post-operatively.
Findings:
- Autopsy revealed diffuse intimal fibromuscular dysplasia affecting arteries supplying the heart, liver, kidneys, and intestines.
- Evidence of recent intestinal, kidney, and liver infarction was observed.
- This case demonstrates the potential for rapid progression and multi-organ involvement in intimal FMD.
Implications:
- Intimal FMD can manifest as a diffuse and rapidly progressive condition.
- This case underscores the importance of considering FMD in patients with unexplained vascular events.
- Emerging treatments for restenosis prevention after angioplasty may offer therapeutic avenues for intimal FMD.