Fibromuscular dysplasia differential diagnosis: Difference between revisions

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|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
| colspan="7" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
| colspan="8" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Clinical manifestations'''
! colspan="6" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para-clinical findings
! colspan="6" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Para-clinical findings
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
| colspan="1" rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Gold standard'''
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|-
|-
| colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
| colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |'''Symptoms'''
! colspan="3" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
! colspan="4" rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination    
|-
|-
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab Findings
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
|-  
|-  
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptom 1
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Headache
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptom 2
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Heart attack/
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptom 3
chest pain
!Other symptoms
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Cerebrovascular accident
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical exam 1
!Systemic symptoms
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical exam 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Hypertension
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical exam 3
!Chest pain     
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab 1
! colspan="1" rowspan="1" style="background: #4479BA; color: #FFFFFF; text-align: center;" |motor or/and sensory deficit
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Arterial occlusion or/and rupture
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Metabolic disturbances
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 1
(Hyperlipidemia/Hyperglycemia)
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 2
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Inflammatory biomarkers
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging 3
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Specific Lab Data
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Angiography
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |CTA/MRA
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Duplex
ultrasound
|-
|Fibromuscular
 
dysplasia
| +
| +/-
| +/-
| -
| +/-
| +/-
| +/-
| +/-
| -
| -
|
| +
| +
| +
|Angiography
|
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Atherosclerotic Vascular Disease
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Atherosclerotic Vascular Disease
| style="background: #F5F5F5; padding: 5px;" |Heart attack
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |Stroke
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |[[Peripheral artery occlusive disease]]
| style="background: #F5F5F5; padding: 5px;" | +
|
|<nowiki>-</nowiki>
| style="background: #F5F5F5; padding: 5px;" |[[Chest pain]]
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |motor or/and sensory deficit
| +
| style="background: #F5F5F5; padding: 5px;" |[[intermittent claudication]]
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |Hyperlipidemia
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |Hyperglycemia
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Angiography
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |CT scan or MRI
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |[[Duplex ultrasound]]
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |Angiography
| style="background: #F5F5F5; padding: 5px;" |Angiography
| style="background: #F5F5F5; padding: 5px;" |Generally older than 60 yrs of age
| style="background: #F5F5F5; padding: 5px;" |Generally older than 60 yrs of age
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Takayasu Arteritis
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Takayasu Arteritis
| style="background: #F5F5F5; padding: 5px;" |Headache
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |Malaise
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |Artheralgias and fever
| style="background: #F5F5F5; padding: 5px;" | +/-
|
|<nowiki>+</nowiki>
| style="background: #F5F5F5; padding: 5px;" |Hypertension
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |Absent or diminished pulses
| +/-
| style="background: #F5F5F5; padding: 5px;" |Focal neurologic deficits
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |Elevated ESR and CRP
| style="background: #F5F5F5; padding: 5px;" | +/-
| style="background: #F5F5F5; padding: 5px;" |Leukocytosis
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |Normochromic anemia
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |Color Doppler ultrasonography
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |CT helical scanning angiography
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |MRA
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Female, younger than 40 yrs of age, hypertension, absent or decreased pulses, bruits, murmur of aortic regurgitation
| style="background: #F5F5F5; padding: 5px;" |Female, younger than 40 yrs of age, hypertension, absent or decreased pulses, bruits, murmur of aortic regurgitation
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Moyamoya disease
| style="background: #DCDCDC; padding: 5px; text-align: center;" |Moyamoya disease
| style="background: #F5F5F5; padding: 5px;" |Asymptomatic/variable
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |TIA/ CVA
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |headaches/ dizziness/or seizures
| style="background: #F5F5F5; padding: 5px;" | +
|
| style="background: #F5F5F5; padding: 5px;" | -
|
|
| style="background: #F5F5F5; padding: 5px;" |Non-specific
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |Motor or/and Sensory deficit
| style="background: #F5F5F5; padding: 5px;" |+
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" | -
| style="background: #F5F5F5; padding: 5px;" |Electroencephalography
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Head CT/Brain MRI
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |Transcranial Doppler ultrasonography
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |MRA
| style="background: #F5F5F5; padding: 5px;" | +
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |Children with MMD often exhibit abnormalities on EEG. The most characteristic is the "rebuild-up" phenomenon following hyperventilation.  
| style="background: #F5F5F5; padding: 5px;" |Children with MMD often exhibit abnormalities on EEG. The most characteristic is the "rebuild-up" phenomenon following hyperventilation.  
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!
!
!Physical exam 1
!Physical exam 1
!
! colspan="1" rowspan="1" |Physical exam 2
! colspan="1" rowspan="1" |Physical exam 2
!Physical exam 3
!Physical exam 3
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|
|
| style="background: #F5F5F5; padding: 5px;" |Non-specific S&S of arterial rupture
| style="background: #F5F5F5; padding: 5px;" |Non-specific S&S of arterial rupture
|
| style="background: #F5F5F5; padding: 5px;" |Non-specific S&S intestinal rupture
| style="background: #F5F5F5; padding: 5px;" |Non-specific S&S intestinal rupture
| style="background: #F5F5F5; padding: 5px;" |Non-specific S&S uterine  rupture
| style="background: #F5F5F5; padding: 5px;" |Non-specific S&S uterine  rupture
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|
|
| style="background: #F5F5F5; padding: 5px;" |Non-specific S&S of Arterial occlusion or/and rupture
| style="background: #F5F5F5; padding: 5px;" |Non-specific S&S of Arterial occlusion or/and rupture
|
| style="background: #F5F5F5; padding: 5px;" |Abdominal distention
| style="background: #F5F5F5; padding: 5px;" |Abdominal distention
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |
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|
|
| style="background: #F5F5F5; padding: 5px;" |Acute hypertension
| style="background: #F5F5F5; padding: 5px;" |Acute hypertension
|
| style="background: #F5F5F5; padding: 5px;" |Hole in nasal septum
| style="background: #F5F5F5; padding: 5px;" |Hole in nasal septum
| style="background: #F5F5F5; padding: 5px;" |
| style="background: #F5F5F5; padding: 5px;" |

Revision as of 15:34, 30 August 2018

Fibromuscular dysplasia Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohsen Basiri M.D.

Overview

Fibromuscular dysplasia must be differentiated from other diseases that present clinical features of multisystem involvement, hypertension, 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 is not considered in the differential diagnosis of a patient’s symptoms because of under-recognition of this disorder, as well as the mistaken belief that FMD is predominately a rare 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 and 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]

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 dissections, 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 presentations of this disease are ischemic cerebrovascular events, and hemorrhagic stroke.[5]

Preferred Table

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging
Headache Heart attack/

chest pain

Cerebrovascular accident Systemic symptoms Hypertension Chest pain motor or/and sensory deficit Arterial occlusion or/and rupture Metabolic disturbances

(Hyperlipidemia/Hyperglycemia)

Inflammatory biomarkers Specific Lab Data Angiography CTA/MRA Duplex

ultrasound

Fibromuscular

dysplasia

+ +/- +/- - +/- +/- +/- +/- - - + + + Angiography
Atherosclerotic Vascular Disease - + + - +/- + +/- +/- + - +/- +/- +/- Angiography Generally older than 60 yrs of age
Takayasu Arteritis + +/- +/- + +/- +/- +/- +/- - + + + + Female, younger than 40 yrs of age, hypertension, absent or decreased pulses, bruits, murmur of aortic regurgitation
Moyamoya disease + - + - + + - + + + Children with MMD often exhibit abnormalities on EEG. The most characteristic is the "rebuild-up" phenomenon following hyperventilation.
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 Gold standard Additional findings
Ehlers-Danlos Type IV Arterial rupture Intestinal

rupture

Uterine rupture Non-specific S&S of arterial rupture Non-specific S&S intestinal rupture Non-specific S&S uterine rupture Sequence and deletion/duplication testing of the COL3A1 gene Analysis of type III procollagen from fibroblasts culture Younger than 40 yrs of age, small body habitus, characteristic facial appearance, translucent skin, easy bruising, hypermobile joints
Segmental arterial mediolysis Asymptomatic Abdominal or flank pain Intensive headache/ CVA Non-specific S&S of Arterial occlusion or/and rupture Abdominal distention Rapidly decreasing hematocrit Hematuria Acid/base disturbances CTA Angiography MRA Histology A rare conditions in the elderly
Cocaine vasculitis Arthralgias Skin lesions constitutional symptoms: fever, night sweats, weight loss, myalgias Acute hypertension Hole in nasal septum Urine toxicology ECG may show ST-segment elevations MPO-ANCA/PR3-ANCA Urine toxicology Men with fewer cardiovascular risk factors

References

  1. 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)
  2. Schievink WI, Limburg M. Angiographic abnormalities mimicking fibromuscular dysplasia in a patient with Ehlers-Danlos syndrome, type IV. Neurosurgery. 1989;25:482–483.
  3. 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)
  4. 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)
  5. 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)

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