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thromboangiitis obliterans; Buerger's disease
Inflammatory occlusive vascular disorder affecting small & medium size arteries & veins in the upper & lower extremities. Cerebral, visceral & coronary arteries may also be involved.
Etiology:
1) unknown
2) association with cigarette smoking
Epidemiology:
1) most frequent in men 30-50 years [3]
2) more common in Asians & eastern Europeans
Pathology:
1) distal extremity ischemia, digital ischemia
2) neutrophil infiltration of small & medium size arteries & veins affecting vessels of distal limbs
3) cerebral arteries, visceral arteries & coronary arteries may also be involved
4) internal elastic lamina is preserved
5) thrombosis may occur in vascular lumen
6) granuloma formation supersedes neutrophil invasion
7) perivascular fibrosis & recanalization in later phases
Genetics: increased incidence of HLA-B5 & A9 in patients
Clinical manifestations:
1) claudication of affected extremity
a) confined to calves & feet or forearms & hands
b) affects distal vessels, digits
c) ulceration &/or gangrene [3]
2) Raynaud's phenomenon
3) migratory superficial vein thrombophlebitis
4) normal brachial artery & popliteal artery pulses
5) radial artery, ulnar artery or tibial artery pulses may be absent
- positive Allen test
Laboratory:
- inflammatory markers normal
- biopsy of affected vessel (see pathology)
Radiology:
- arteriography
a) distal segmental vascular disease
b) corkscrew collateral formation at sites of occlusion
Differential diagnosis:
- systemic vasculitis* present with elevated inflammatory markers
- scleroderma: thickened skin (sclerodactyly), positive ANA
- endocarditis: positive blood cultures, elevated inflammatory markers. unlikely with normal transesophageal echocardiogram [3]
* polyarteritis nodosa, cryoglobulinemia, microscopic polyangiitis, Wegener's granulomatosis
Management:
1) no specific treatment
2) abstain from smoking
3) arterial bypass of larger vessels
4) antibiotics may be useful
5) anticoagulants & glucocorticoids are not helpful
6) amputation of gangrenous extremities
Related
claudication
Raynaud's phenomenon
General
arteritis
thrombophlebitis (includes superficial venous thrombosis)
chronic vascular disease (chronic vasculopathy)
References
- Harrison's Principles of Internal Medicine, 14th ed.
Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1400-01
- Medical Knowledge Self Assessment Program (MKSAP) 11, American
College of Physicians, Philadelphia 1998
- NEJM Knowledge+
- Piazza G, Creager MA.
Thromboangiitis obliterans.
Circulation. 2010 Apr 27;121(16):1858-61.
PMID: 20421527 PMCID: PMC2880529 Free PMC article. Review.
- Olin JW
Thromboangiitis obliterans. (Buerger's Disease)
N Engl J Med 2000. 343:864 Sept 21
PMID: 10995867
https://www.nejm.org/doi/pdf/10.1056/NEJM200009213431207
- Rivera-Chavarria IJ, Brenes-Gutierrez JD.
Thromboangiitis obliterans (Buerger's disease).
Ann Med Surg (Lond). 2016 Mar 29;7:79-82.
PMID: 27144003 PMCID: PMC4840397 Free PMC article. Review.