Thrombangiitis obliterans I73.1

Authors: Prof. Dr. med. Peter Altmeyer, Prof. Dr. med. Martina Bacharach-Buhles

All authors of this article

Last updated on: 07.05.2022

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Synonym(s)

Billroth von Winiwarter disease; Citizen's disease; Citizen's Disease; Citizen's Syndrome; endangiitis obliterans; Endarteritis; Leo Citizen's Disease; Morbus Winiwarter-Citizen; TAO; von-Winiwarter-Buerger disease; Winiwarter citizen M.

History
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Friedländer, 1876; von Winiwarter, 1879; Buerger, 1908

Definition
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Inflammatory, non-atherosclerotic (autoimmunological?), segmental, mostly obliterating panarteriitis of the small and medium-sized arteries and veins, which mainly affects young male smokers.

Occurrence/Epidemiology
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Worldwide distribution with higher prevalence in Orient, India, Southeast Asia, Eastern Europe. Estimated incidence of 6.8/100,000 white males aged 22-44 years. In Israel, the incidence is 1:5000 /year.

Etiopathogenesis
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Unknown, of pathogenetic importance is inhaled cigarette smoking (hypersensitivity to nicotine). Deposition of immunoglobulins and complement in the vascular intima.

Possible immune reaction against a nicotine-induced self-antigen in the vascular wall. Rheumatic fever caused by streptococci is also discussed.

Autoimmune mechanisms are questionable.

There are ethnic predispositions and HLA associations (HLA-A9, HLA-B5).

Pathogenetically, anti-elastin antibodies indicate an increased cell-mediated immunity to collagen.

Manifestation
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Mostly occurring in men (smokers in 98% of cases), mainly between the ages of 17 and 44. The male/female ratio seems to change in recent years to the disadvantage of women (earlier figures proved a ratio of 100:2.5; more recent figures prove a ratio of m:w=3:1).

Localization
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Especially lower extremities (infrapopliteal).

Clinical features
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Smoker's history!

Selective infestation of distal arteries and veins, recurrent, mostly superficial thrombophlebitis, thrombophlebitis migrans, later mainly acral necroses without healing tendency.

Integument: Often livid or cyanotic coloration of the affected acra. Initially red, 2.0-5.0 cm large, isolated or confluent plaques and nodules painful spontaneously and under pressure; also only palpable painful indurations. Often weeping, occasionally encrusted, painful ulcers with hyperkeratotic margins. Necroses of the cap and acral osteolysis in advanced stages.

General: Paresthesia (40%), sensation of cold (60%), cyanosis (40%), claudication in the instep, sole (sole claudication - often misdiagnosed as an orthopaedic condition)- or calf area (70%), ischaemic pain at rest (50%), Accompanying superficial phlebitis (40%), early trophic disturbances and necroses on nail fold, acra (cupped necroses) or the back of the foot (50%), episodes of phlebitis saltans/migrans (27%), Raynaud's phenomenon. Rare cerebral or abdominal involvement.

In individual cases necrotizing sialometaplasia.

Histology
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Inflammatory wall infiltration, intimate thickening of the arteries, possible occlusion. Segmental panangiitis of middle and small arteries and veins of the lower extremities. In fresh lesions early organized, cell-rich thrombi with microabscesses of giant cells, epithelioid cells and leukocytes. Lymphocytic-fibroblastic infiltration of all vascular wall layers. In older thrombi usually signs of revascularization of varying degrees of progress.

Diagnosis
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Smoker's history! Hospital. Colour duplex.

MR angiography: absence of plaques in the proximal arteries. Sudden or gradual narrowing of the distal arteries ("tapering" type), segmental occlusions ("skip" lesions), corkscrew-like or root-like small collaterals or revascularized vessels(corkscrew collaterals). Often striking bilateral symmetry.

Differential diagnosis
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  • Cholesterol embolisms: Men > 60 years; general arteriosclerosis; mostly signs of livedo racemosa with jagged ulcers.
  • Erythromelalgia: attacks of flat redness; characteristic is the cold water test = pain stops under cold water, after rewarming new pain.
  • Perniones: no signs of AVK; mostly women are affected
  • Erythema nodosum: attacks of high pain; mostly general symptoms e.g. fever, signs of infection; no AVK; women are more frequently affected than men.
  • Systemic polyarteritis nodosa: signs of systemic disease; weight loss; diffuse myalgia or muscle weakness; hypertension with diastole > 90 mm Hg; polyneuropathy; renal insufficiency; aneurysms or occlusions of abdominal arteries; skin infestation (not obligatory): livedo, painful papules and nodules, especially in the lower extremities.
  • Chilblain lupus: mostly acral plaques and nodules; almost exclusively in women; no AVK; signs of lupus erythematosus.

General therapy
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Abstaining from nicotine is the first measure! In addition, reduction of other risk factors by focus rehabilitation ( streptococcus infections), avoidance of cold.

Notice!

Warm baths are contraindicated due to the increased O2 requirement of the peripheral vessels!

External therapy
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The disease is associated with the symptoms of an arterial occlusive disease. S.u. thrombophlebitis; occlusive arterial disease, acute; acute vascular occlusion; ulcer; gangrene. S.a.u. Wound treatment.

Internal therapy
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Possibly short-term glucocorticoids in high doses such as prednisolone 100-150 mg/day (e.g. Decortin H Tbl.). Vasodilating substances such as prostaglandins (e.g. Prostavasin), prostacyclin derivatives such as iloprost (ilomedin) or pentoxifylline (e.g. Trental) are controversial in their effects.

Operative therapie
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Border strand blockage and sympathectomy are temporarily effective. Ultima ratio is amputation (not rare!).

Progression/forecast
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Quoad vitam favourable; unfavourable with regard to the preservation of the limb.

Note(s)
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Endangitis obliterans is a definite clinical feature due to the inflammation of the vessel wall leading to obliteration of peripheral vessels. It belongs to the group of vasculitides, even if it is usually not listed there.

Case report(s)
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  • The 35-year-old patient (smoker since the 16th LJ; 30-40 cigarettes/day), noticed painful lumps on both lower legs and on the soles of his feet for several months. The distinct pain was the leading clinical symptom.
  • Findings: Acral acrocyanosis of the distal extremity; deep cyanotic discoloration of the right digestive tract IV. On the right sole of the foot two approx. 2 x 2 cm large, blurred, pressure painful, red plaques appear. Red, flat, painful indurations were found in the area of the lat. malleolus of the left foot. Reduced occlusion pressures of the right dorsalis pedis (70 mm Hg) and the left tibialis post. (85 mm Hg) at a system pressure of 130/85 mm Hg. Duplex sonography revealed stenosis of the tibialis limb ant. and post. Formation of collaterals (corkscrew collaterals).
  • Histologically, a medium-sized artery with occluding granulation tissue could be detected by deep excision biopsy in serial cuts. In the center of the thrombus signs of revascularization.

Literature
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  1. Buerger L (1908) Thrombangitis obliterans: a study of the vascular lesion leading to presenile spontaneous gangrene. On the J med Sci 136: 567-580
  2. Diehm C, Stammler F (2001) Thromboangiitis obliterans (Buerger's disease). N Engl J Med 344: 230-231
  3. Friedländer C (1876) Arteriitis obliterans. Zentralbl med Wiss (Berlin) 1876: 14
  4. Franke W, Schulte KW, Ruzicka T, Krutmann J (2000) Thromboangiitis obliterans. dermatologist 51: 604-611
  5. Fujii Y et al (2007) Images in cardiovascular medicine. Corkscrew collaterals in thromboangitis obliterans (Buerger's disease). Circulation 116:e539-540
  6. Hoeft D et al (2004) Nodular erythema of both feet. dermatologist 55: 874-876
  7. Jiménez-Ruiz CA et al (2006) Smoking characteristics and cessation in patients with thromboangiitis obliterans. Monaldi Arch Chest Dis 2006 65: 217-221
  8. Lee T et al (2003) Immunobiological analysis of arterial tissue in Buerger's disease. Eur J Vasc Endovasc Surg 25: 451-457
  9. Guidelines on diagnostics and therapy in vascular surgery. Published by the board of directors of Dt. Ges. f. Gefäßchirurgie; Deutscher Ärzteverlag, Cologne 1998
  10. Olin JW (2001) Thromboangiitis obliterans (Buerger's disease). N Engl J Med 343: 864-869
  11. Paraskeva's AI, Liapis CD et al (2007) Thromboangiitis obliterans (Buerger's disease): searching for a therapeutic strategy. Angiology 58: 75-84
  12. Takanashi T, Horigome R et al (2007) Buerger's disease manifesting nodular erythema with livedo reticularis. Internal med 46: 1815-1819
  13. v. Winiwarter F (1879) About a peculiar form of endocarditis and endophlebitis with gangrene of the foot. Arch klin Chir Berlin 23: 202-226

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