Vasculitis

The collective term vasculitis diseases are summarized, which are due to autoimmune processes - most commonly an allergy type 3 - inflammation of arteries, arterioles, capillaries, venules and veins occurs, whereby the supplied organs themselves can be damaged as a consequence. The causes are not yet clear. Currently, genetic suspects in combination with environmental factors such as infections with the bacterium Staphylococcus aureus, or hepatitis virus.

Previously led vasculitis within a few months to death, but today it can be brought about by treatment with immunosuppressants in remission, making the disease, however, is not defeated: Since the immune system is derailed to change and the causes for it are not decrypted as before, the also means can not be eliminated, it can always come back to a relapse.

In contrast, vasculopathy are a group of primarily non-inflammatory vascular disease of various causes that lead to a partial or complete blockage of a vessel. In practice, both types of diseases are, however, linguistically often not clearly separated.

Classification

Primary vasculitis

Primary vasculitis vasculitis are no known underlying disease. They are after the Chapel Hill Consensus Conference on the size of the affected vessels and depending on whether they have ANCA antibodies or not, classified as follows:

Primary vasculitis of small vessels

ANCA -associated vasculitis

• Microscopic polyarteritis (abbreviation: MPA or MPAN )

Non -ANCA -associated vasculitis For the non -ANCA - associated vasculitis of the small vessels, there are immune complex - and Komplementablagerungen, so it involves primary Immunkomplexvaskulitiden.

• Henoch-Schönlein purpura
• Vasculitis in essential (ie, idiopathic ) cryoglobulinemia
• Cutaneous leukocytoclastic angiitis

Immune complex vasculitis of the small vessels occur also in other diseases before (eg, serum sickness, collagen ). But then it is not primary but secondary to vasculitis in the course of a known underlying disease.

Primary vasculitis of medium vessels

• Polyarteritis nodosa (PAN or CPAN ) than ANCA -negative vasculitis in virtually all organs including neurological, cutaneous and renal symptoms, and occasionally secondary to hepatitis B
• Kawasaki syndrome

Primary vasculitis of large vessels

• Giant cell arteritis ( temporal arteritis also, cranial arteritis or Horton disease called )
• Takayasu 's arteritis as ANCA -negative vasculitis especially the aorta and its main branches

2012 has been defined as the fourth group, the vasculitis of medium-sized vessels. It includes Behçet's disease.

Secondary vasculitis

The secondary vasculitis occur in autoimmune disorders and infectious diseases (eg AIDS, syphilis) or are induced by medication.

The acute vascular organ rejection after a kidney transplant is one of them, cause here are synthesized by the recipient IgG antibodies against alloantigens of the epithelial cells of the graft.

Similarly, secondary vasculitis after ingestion of cocaine and ergot alkaloids and some medicinal plants may occur.

Unclassified vasculitis

Unclassified vasculitis are the thromboangiitis obliterans, the Behçet 's syndrome and the isolated CNS angiitis.