Endangiitis obliterans

from Wikipedia, the free encyclopedia
Classification according to ICD-10
I73.1 Thrombangiitis obliterans [Endangiitis von-Winiwarter-Buerger]
ICD-10 online (WHO version 2019)
Buerger's disease

The thromboangiitis obliterans or thromboangiitis obliterans is a segmental vascular inflammation ( vasculitis ) of small and medium-sized arteries and veins mainly the lower extremity. Internal organs are usually not affected. Synonyms are Winiwarter-Buergersche disease , Crohn obliterans , obliterans-Buerger's disease or Buerger's disease , named after Felix von Winiwarter and Leo Buerger .

It usually affects young men under the age of 40 with heavy nicotine consumption (75 percent of those affected). Rather rare in Western Europe with the exception of the Mediterranean region, strongest prevalence in India, Korea and Japan. Life expectancy is usually not reduced.

etiology

The cause of endangiitis obliterans is not yet known, but an interplay between genes (especially HLA B5 and A9) and exogenous pollutants (noxae) ( nicotine ) is postulated . Also autoantibodies are discussed in the making. The nicotine leads to an excessive toning of the veins and thus the (blood) flow becomes more important as a pathological factor. There seems to be a genuine readiness for inflammation.

Symptoms and Diagnosis

Usually, the affected extremity feels cold in combination with pain at rest, similar to that of peripheral arterial occlusive disease . Raynaud's syndrome of the affected extremity and wandering superficial inflammation of the veins ( thrombophlebitis / phlebitis ) are also typical . A bluish discoloration of the outer extremities accompanied by pain to the touch can also be a sign of the disease. On the tips of the fingers and toes, necroses , gangrene and trophic disorders of the nails up to acroosteolysis can be found . The diagnosis is usually made on the basis of the clinical course, angiography, and patho-histological examination of the affected vessels.

Differential diagnosis

In particular, classic peripheral arterial occlusive disease , arterial embolism , venous insufficiency and other vasculitis must be excluded.

pathology

Initially there is a mixed inflammatory infiltrate of lymphocytes , granulocytes and plasma cells in all layers of the vessel wall . The inflammation leads to endothelial damage with secondary deposition of thrombotic material and necrosis of the tunica media of the vessels and ultimately to closure of the affected vessel. The thrombus can then be recanalized. The vascular wall usually fibrosis .

therapy

Immediate cessation of nicotine can lead to a standstill of the disease. Patients who continue to smoke have a poor prognosis. Infusion therapy with prostaglandin E1 and prophylactic therapy with acetylsalicylic acid are advisable. A sympathectomy may help. In the case of extensive necrosis , amputations may be necessary. The amputation rate of the affected limb is around 30 percent.

In the context of course uncontrolled observational studies an application could successive Immunadsorptionszyklen a strong decrease in the disease activity for clinical symptoms, opioid consumption and ischemia show -Ereignissen.

Initial description

  • F. v. Winiwarter: About a peculiar form of endarteritis and endophlebitis with gangrene of the foot. In: Arch Klin Chir 23/1879 p. 202.
  • L. Buerger: Thrombo-Angiitis Obliterans: A study of the vascular lesions leading to presenile spontaneous gangrene. In: Am J Med Sci 136/1908 p. 567.

literature

Web links

Commons : Endangiitis obliterans  - Collection of images, videos and audio files

Individual evidence

  1. ↑ PF Klein-Weigel, C. Köning, A. Härtwig, K. Krüger, B. Gutsche-Petrak, S. Dreusicke, U. Thieme, K. Enke-Melzer, B. Urbach, J. Kron: Immunoadsorption in Thrombangiitis Obliterans - A Promising Therapeutic Option. Results of a Consecutive Patient Cohort Treated in Clinical Routine Care .