Vasculitis
Classification according to ICD-10 | |
---|---|
I77.6 | Arteritis, unspecified |
L95.- | Vasculitis not elsewhere classified and limited to the skin |
ICD-10 online (WHO version 2019) |
The collective term vasculitis ( inflammation of the blood vessels ) covers diseases in which autoimmunological processes - most commonly a type 3 allergy - lead to inflammation of arteries , arterioles , capillaries , venules and veins , which consequently damage the organs supplied become. The causes are not clear. Genetic factors in combination with environmental influences such as infections with the bacterium Staphylococcus aureus or the hepatitis virus are suspected.
In the past, vasculitides led to death within a few months, but today they can be brought into remission through therapy with immunosuppressive drugs , which does not defeat the disease, however: Since the immune system is permanently derailed and the causes for it are still not deciphered and therefore also cannot be remedied, a relapse can occur at any time .
In contrast to this, vasculopathies are a group of primarily non-inflammatory vascular diseases of different causes that lead to a partial or complete occlusion of a vessel. In practice, however, the two types of disease are often not clearly separated from one another by language.
classification
Primary vasculitis
Primary vasculitis is vasculitis with no known underlying disease. They are after the Revised Chapel Hill Consensus Conference nomenclature (2012) on the size of the vessels concerned and depending on whether they ANCA - antibodies classified or may not have, as follows:
Primary small vessel vasculitis
ANCA-associated vasculitis
- Granulomatosis with polyangiitis (old name: Wegener's granulomatosis ) with vasculitis mainly of the small arteries of the kidneys and lungs
- Eosinophilic granulomatosis with polyangiitis (old name: Churg-Strauss syndrome )
- Microscopic polyarteritis (abbreviation: MPA or mPAN)
Non-ANCA-Associated Vasculitides
The non-ANCA-associated vasculitides of the small vessels show immune complex and complement deposits, so these are primary immune complex vasculitides.
- IgA vasculitis ( Henoch-Schönlein purpura )
- Vasculitis in essential (ie idiopathic) cryoglobulinemia
- Anti-GBM Disease (Glomerular Basement Membrane)
- Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)
Primary middle vessel vasculitis
Primary large vessel vasculitis
- Giant (formerly temporal arteritis , arteritis cranial or Crohn Horton called)
- Takayasu arteritis as ANCA-negative vasculitis, especially of the aorta and its main branches
Primary vasculitis of variable vessel size
Primary organ vasculitis
- cutaneous leukocytoclastic angiitis
- primary CNS vasculitis and the like a.
Secondary vasculitis
Secondary vasculitides occur in autoimmune diseases (e.g. systemic lupus erythematosus ) and infectious diseases (e.g. AIDS , syphilis ) or are medically induced.
Acute vascular organ rejection after a kidney transplant is one of them, the cause of this being the IgG antibodies synthesized by the recipient against alloantigens of the epithelial cells of the transplant.
Secondary vasculitis can also occur after ingestion of cocaine and ergot alkaloids as well as some medicinal plants .
See also
literature
- S1- guideline vasculitis of the German Society for Child and Adolescent Medicine e. V. (DGKJ). In: AWMF online (as of 2013)
- S1 guideline on cerebral vasculitis and cerebral involvement in systemic vasculitis and underlying rheumatic diseases
- Gerd Herold: Internal Medicine. 2016. ISBN 978-3-9814660-5-8 .
- ARTHRITIS & RHEUMATISM Vol. 65, No. 1, January 2013, pp 1–11: 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides
Web links
- Description of the disease vasculitis of the German Society for Rheumatology
- European Vasculitis Study Group (English)
- Interdisciplinary Vasculitis Center , information for patients and relatives (German)
Individual evidence
- ↑ Alphabetical directory for the ICD-10-WHO Version 2019, Volume 3. German Institute for Medical Documentation and Information (DIMDI), Cologne, 2019, p. 926