Fibromuscular dysplasia differential diagnosis: Difference between revisions
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fibromuscular dysplasia in a patient with Ehlers-Danlos syndrome, type | fibromuscular dysplasia in a patient with Ehlers-Danlos syndrome, type | ||
IV. Neurosurgery. 1989;25:482–483.</ref> | IV. Neurosurgery. 1989;25:482–483.</ref> | ||
*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]]. | *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. | [[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. | ||
Revision as of 05:33, 16 June 2018
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Fibromuscular dysplasia Microchapters |
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Diagnosis |
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Treatment |
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ASA/ACCF/AHA Guideline Recommendations |
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Management of Patients With Fibromuscular Dysplasia of the Extracranial Carotid Arteries |
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Risk calculators and risk factors for Fibromuscular dysplasia differential diagnosis |
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.[1]
In general,Atherosclerosis occurs proximally usually involves the proximal segment of the arteries, whereas FMD involves the mid or distal portion of the blood vessel. The "string of beads" appearance is remarkable for FMD; and atherosclerotic disease and FMD can be differentiated radiographically.
- Ehlers-Danlos Type IV may be associated with medial fibroplasia. This differential diagnosis should be considered in patients who have multiple aneurysms in addition to the routine angiographic characteristics of FMD.[2]
- 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. [3]
- Differential diagnosis of FMD Involving the [[[coronary arteries]], are cocaine vasculitis, coronary vasospasm, and atherosclerotic plaque.[4]
- Moyamoya disease (MMD) is a unique disease by bilateral stenosis of the arteries of the circle of Willis and arterial collateral vessels and formation of an abnormal vascular network in the vicinity of the arterial occlusion. The most presenttions of this disease are ischemic cerebovascular evevnts, and hemorrhagic stroke.[5]
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
- ↑ David P. Slovut & Jeffrey W. Olin (2004). "Fibromuscular dysplasia". The New England journal of medicine. 350 (18): 1862–1871. doi:10.1056/NEJMra032393. PMID 15115832. Unknown parameter
|month=ignored (help) - ↑ Schievink WI, Limburg M. Angiographic abnormalities mimicking fibromuscular dysplasia in a patient with Ehlers-Danlos syndrome, type IV. Neurosurgery. 1989;25:482–483.
- ↑ Jeffrey W. Olin, Heather L. Gornik, J. Michael Bacharach, Jose Biller, Lawrence J. Fine, Bruce H. Gray, William A. Gray, Rishi Gupta, Naomi M. Hamburg, Barry T. Katzen, Robert A. Lookstein, Alan B. Lumsden, Jane W. Newburger, Tatjana Rundek, C. John Sperati & James C. Stanley (2014). "Fibromuscular dysplasia: state of the science and critical unanswered questions: a scientific statement from the American Heart Association". Circulation. 129 (9): 1048–1078. doi:10.1161/01.cir.0000442577.96802.8c. PMID 24548843. Unknown parameter
|month=ignored (help) - ↑ Katherine C. Michelis, Jeffrey W. Olin, Daniella Kadian-Dodov, Valentina d'Escamard & Jason C. Kovacic (2014). "Coronary artery manifestations of fibromuscular dysplasia". Journal of the American College of Cardiology. 64 (10): 1033–1046. doi:10.1016/j.jacc.2014.07.014. PMID 25190240. Unknown parameter
|month=ignored (help) - ↑ 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
|month=ignored (help)