Fibromuscular dysplasia differential diagnosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
- Fibromuscular dysplasia must be differentiated from other diseases that present clinical features of multisystem involvement, hepertension,aneurysm, and dissection, such as atherosclerotic vascular disease, vasculitis, and Ehlers-Danlos Type IV.
- There is a significant delay in diagnosis from the first onset of clinical symptoms/signs of 4.4 years in men and 4.1 years in women. There are several possible reasons for such a delay, including the possibility that FMD was not considered in the differential diagnosis of a patient’s symptoms because of under-recognition of this disorder, the mistaken belief that FMD is predominately a disease of young patients, and the fact that many of the signs and symptoms of FMD are nonspecific, such as dizziness, tinnitus, and headaches.
Differentiating X from other Diseases
- Fibromuscular dysplasia must be differentiated from atherosclerotic vascular disease.. Patients with atherosclerosis often have multiple atherosclerotic risk factors, whereas most individuals with FMD are younger and have fewer risk factors.
Atherosclerosis usually involves the ostial or proximal segment of the arteries, whereas FMD involves the middle or distal segment. In addition, the "string of beads" appearance is unique to FMD. Thus, atherosclerotic disease and FMD can typically be distinguished radiographically.
- There have been reports of Ehlers-Danlos Type IV being associated with medial fibroplasia. This should be suspected in patients who have multiple aneurysms in addition to the usual angiographic findings of FMD.
Ehlers-Danlos may occur in the absence of any prior bleeding manifestations. If this disease is suspected, a skin biopsy should be obtained and sent for fibroblast culture.
- Multisystem involvement is observed in both vasculitis and FMD. Those with FMD generally will not have associated anemia, thrombocytopenia, or abnormalities of acute phase reactants. An exception is in the setting of an acute infarction.
Large vessel vasculitis may occur in the absence of changes in acute phase reactants in up to 40% of cases. If histologic proof of FMD or inflammation is not available, distinguishing these entities may at times be difficult because the angiographic appearance can be similar, especially if the intimal fibroplasia is of the multivessel type.
- Segmental arterial mediolysis is a poorly understood condition characterized by spontaneous dissection(s), occlusion, or aneurysm formation, which may be difficult to differentiate from FMD. Similar to FMD, segmental arterial mediolysis is a noninflammatory, nonatherosclerotic arterial disease. A definitive diagnosis of segmental arterial mediolysis requires tissue examination. This lesion of segmental arterial mediolysis is characterized by the vacuolar degeneration of smooth muscle cells.
- Differential diagnosis of FMD Involving the [[[coronary arteries]], are cocaine vasculitis, coronary vasospasm, and atherosclerotic plaque.
- Moyamoya disease (MMD) is a unique disease by bilateral stenosis of the arteries of the circle of Willis and arterial collateral vessels. The most presenttions of this disease are ischemic cerebovascular evevnts, and hemorrhagic stroke.[1]
Preferred Table
| Diseases | Clinical manifestations | Para-clinical findings | Gold standard | Additional findings | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Symptoms | Physical examination | ||||||||||||||
| Lab Findings | Imaging | Histopathology | |||||||||||||
| Symptom 1 | Symptom 2 | Symptom 3 | Physical exam 1 | Physical exam 2 | Physical exam 3 | Lab 1 | Lab 2 | Lab 3 | Imaging 1 | Imaging 2 | Imaging 3 | ||||
| Differential Diagnosis 1 | |||||||||||||||
| Differential Diagnosis 2 | |||||||||||||||
| Differential Diagnosis 3 | |||||||||||||||
| Diseases | Symptom 1 | Symptom 2 | Symptom 3 | Physical exam 1 | Physical exam 2 | Physical exam 3 | Lab 1 | Lab 2 | Lab 3 | Imaging 1 | Imaging 2 | Imaging 3 | Histopathology | Gold standard | Additional findings |
| Differential Diagnosis 4 | |||||||||||||||
| Differential Diagnosis 5 | |||||||||||||||
| Differential Diagnosis 6 | |||||||||||||||
References
- ↑ Dong-Chuan Guo, Christina L. Papke, Van Tran-Fadulu, Ellen S. Regalado, Nili Avidan, Ralph Jay Johnson, Dong H. Kim, Hariyadarshi Pannu, Marcia C. Willing, Elizabeth Sparks, Reed E. Pyeritz, Michael N. Singh, Ronald L. Dalman, James C. Grotta, Ali J. Marian, Eric A. Boerwinkle, Lorraine Q. Frazier, Scott A. LeMaire, Joseph S. Coselli, Anthony L. Estrera, Hazim J. Safi, Sudha Veeraraghavan, Donna M. Muzny, David A. Wheeler, James T. Willerson, Robert K. Yu, Sanjay S. Shete, Steven E. Scherer, C. S. Raman, L. Maximilian Buja & Dianna M. Milewicz (2009). "Mutations in smooth muscle alpha-actin (ACTA2) cause coronary artery disease, stroke, and Moyamoya disease, along with thoracic aortic disease". American journal of human genetics. 84 (5): 617–627. doi:10.1016/j.ajhg.2009.04.007. PMID 19409525. Unknown parameter
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