thromboangiitis obliterans; Buerger's disease
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Introduction
Inflammatory occlusive vascular disorder affecting small & medium size arteries & veins in the upper & lower extremities. Cerebral, visceral & coronary arteries may also be involved.
Etiology
- unknown
- association with cigarette smoking
Epidemiology
Pathology
- distal extremity ischemia, digital ischemia
- neutrophil infiltration of small & medium size arteries & veins affecting vessels of distal limbs
- cerebral arteries, visceral arteries & coronary arteries may also be involved
- internal elastic lamina is preserved
- thrombosis may occur in vascular lumen
- granuloma formation supersedes neutrophil invasion
- perivascular fibrosis & recanalization in later phases
Genetics
increased incidence of HLA-B5 & A9 in patients
Clinical manifestations
- claudication of affected extremity
- Raynaud's phenomenon
- migratory superficial vein thrombophlebitis
- normal brachial artery & popliteal artery pulses
- 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
- distal segmental vascular disease
- 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
- no specific treatment
- abstain from smoking
- arterial bypass of larger vessels
- antibiotics may be useful
- anticoagulants & glucocorticoids are not helpful
- amputation of gangrenous extremities
More general terms
- arteritis
- thrombophlebitis (includes superficial venous thrombosis)
- chronic vascular disease (chronic vasculopathy)
Additional terms
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
- ↑ 3.0 3.1 3.2 3.3 NEJM Knowledge+
Piazza G, Creager MA. Thromboangiitis obliterans. Circulation. 2010 Apr 27;121(16):1858-61. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20421527 PMCID: PMC2880529 Free PMC article. Review.
Olin JW Thromboangiitis obliterans. (Buerger's Disease) N Engl J Med 2000. 343:864 Sept 21 PMID: https://www.ncbi.nlm.nih.gov/pubmed/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: https://www.ncbi.nlm.nih.gov/pubmed/27144003 PMCID: PMC4840397 Free PMC article. Review.