Thrombangiitis obliterans I73.1

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

All authors of this article

Last updated on: 18.09.2022

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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.

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

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Inflammatory, relapsing, non-atherosclerotic (autoimmunological?), multilocular, segmental, mostly obliterating panarteritis of the small and medium-sized arteries and veins, which mainly affects young male smokers (Buerger 2009). The disease may be diagnosed if the clinical manifestations began before the age of 40, the distal arteries (lower leg, hand-foot) are selectively affected and thrombophlebitis saltans is present.

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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.

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Tobacco abuse: The very close relationship of the occurrence of the disease and the disease episodes to a mostly severe tobacco abuse ("no tabacco, no Buerger's disease") is striking. The marked geographic and ethnic prevalence differences suggest genetic factors (there are associations with HLA-A9, HLA -B5), which cause a particular susceptibility to the disease in the presence of active and/or passive smoking behavior (nicotine-induced intrinsic antigen in the vascular wall).

Genetics: MICA genotyping data in 81 Japanese patients with Takayasu arteritis, 38 Japanese patients with thrombangiitis oblterans, and 160 healthy Japanese controls showed that polymorphisms in the MICA gene were significantly associated with Takayasu arteritis or thrombangiitis obliterans (Kimura A et al 1998) .

Immunology: Deposits of immunoglobulins and complement in the vascular intima are detectable, but only in fresh lesions. They are concentrated around the lamina elastica interna, which is altered in the course of this inflammatory event but, in contrast to many other vasculitides, remains intact (Klein-Weigel et al. 2014). Streptococcal initiation of the process is also discussed. Pathogenetically, anti-elastin antibodies indicate increased cell-mediated immunity to collagen.

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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).

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Especially lower extremities (infrapopliteal).

Clinical features
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Smoking history!

Selective involvement of distal arteries and veins, recurrent, mostly superficial thrombophlebitis, thrombophlebitis saltans, later mainly acral necrosis without healing tendency.

Integument: Frequent livid or cyanotic coloration of the affected acras. Initially, red, isolated or confluent plaques and nodules, 2.0-5.0 cm in size, painful spontaneously and on pressure; also palpable painful indurations only. Often weeping, occasionally crusted, painful ulcers with hyperkeratotic margins. Dome necrosis and acral osteolysis in advanced courses.

General: Paresthesias (40%), cold sensation (60%), cyanosis (40%), claudication in instep, plantar (plantar claudication-often misdiagnosed as an orthopedic condition)or calf (70%), ischemic rest pain (50%), concomitant superficial phlebitis (40%), early trophic disturbances and necrosis at nail fold, acral (dome necrosis ), or dorsum of foot (50%), episodes of phlebitis saltans/migrans (27%), Raynaud's phenomenon. Rarely cerebral or abdominal involvement.

In isolated cases, necrotizing sialometaplasia.

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ESR: normal

Increase of pro- and anti-inflammatory cytokines:TNF-α, IL-1β, IL-4, IL-17, IL-23- in plasma (compared to healthy control pesons with and without nicotine abuse).

Detection of antibodies against endothelial cells, elastin as well as collagen. This constellation supports the immune hypothesis of TAO (Klein-Weigel et al. 2014).

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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.

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Smoking history!

Clinic (smoking history, onset before 40 years of age, involvement of distal arteries, thrombophletitis saltans-absent in 40% of cases).

Color duplex: signs of AVK

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

Capillary microscopy: The importance of capillary microscopy is mainly in the differential diagnostic workup. TAO shows nonspecific capillary morphological changes,elongations, capillary hemorrhages and primarily rarefaction of capillaries (Klein-Weigel C et al. 2014).

Differential diagnosis
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Cholesterol embolism: men > 60 years; general arteriosclerosis; mostly signs of livedo racemosa with jagged ulcers.

Erythromelalgia: seizure-like, extensive redness; characteristic is the cold-water test = siesta of pain under cold water, after rewarming renewed pain.

Perniones: no signs of AVK; mostly women are affected

Erythema nodosum: highly painful attacks; mostly general symptoms e.g. fever, signs of infection; no AVC; women are affected more often than men.

Systemic polyarteritis nodosa: signs of systemic disease; weight loss; diffuse myalgias or muscle weakness; hypertension with diastole > 90 mm Hg; polyneuropathy; renal insufficiency; aneurysms or occlusions of abdominal arteries; skin involvement (not obligatory): Livedo, painful papules and nodules especially of lower extremity.

Chilblain's lupus: mostly acrally localized plaques and nodules; almost exclusively in women; no AVC; signs of lupus erythematosus.

General therapy
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Nicotine renunciation is the first measure! In addition, reduction of other risk factors by focus sanitation (streptococcal infections), avoidance of exposure to cold.

Reminder. Warm baths are contraindicated due to the increased O2 demand 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.).

Prostaglandins: The benefit of several weeks of therapy with prostanoids, predominantly the prostacyclin analogue iloprost, was elaborated in two multicenter studies and confirmed in a more recent paper in comparison to sympathectomy (Klein-Weigel C et al. 2014). Treatment in this setting is usually 20 μg/day for five to six hours over three to four weeks, repeated if necessary.

Other vasodilators such as pentoxifylline (e.g., Trental) are controversial in their efficacy.

Operative therapie
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When critical limb ischemia (CLI) occurs in patients with thromboangiitis obliterans (TAO), smoking cessation alone is often insufficient to relieve rest pain and promote wound healing. Accordingly, complementary measures are required to restore adequate blood flow necessary for limb salvage. Percutaneous transluminal angioplasty may be considered a technically feasible and potentially effective treatment for patients with TAO and as a last resort for limb salvage when other options have failed. However, reintervention may be required, especially in patients who continue to smoke (Modaghegh MS et al 2018).

Alternative: Borderline cord blockade and sympathectomy are temporarily effective.

Ultima ratio is amputation (not uncommon!).

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The mortality of patients with TAO is not increased because the coronary and cerebral pathways are not affected. Amputations are required in up to 75% of patients with the disease, with minor amputations predominating. Major amputation rate > 30% (Börner C et al 1998). (Partial) limb loss in multiple extremities is not uncommon. The high amputation rate in still relatively young patients will burden the health care system with high disease-related costs and social consequences of TAO. Socio-medical significance of the disease: frequent loss of employment, early retirement, social isolation and high rates of separation and divorce (Börner C et al. 1998).

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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 LJ 16; 30-40 cigarettes/day), noticed painful nodules on both lower legs as well as on the soles of his feet for several months. The marked pain was the leading clinical symptom.

Findings: Acral acrocyanosis of the distal extremity; deep cyanotic discoloration of dig. IV r. On the sole of the right foot, two red plaques approximately 2 x 2 cm in size, indistinctly circumscribed, with pressure pain. Red, two-dimensional, painful indurations were detectable in the area of the malleolus lat. of the left foot. Decreased occlusion pressures of the right dorsalis pedis artery (70 mm Hg) and the left tibial artery post. (85 mm Hg) with a systemic pressure of 130/85 mm Hg. Duplex sonography revealed evidence of stenosis of the left tibial artery ant. and post. Formation of collaterals (corkscrew collaterals).

Histologically, deep excisional biopsy in serial sections demonstrated a medium-sized artery with occluding granulation tissue. In the center of the thrombus signs of revascularization.

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