Systemic vasculitis is a group of diseases that lead to vasculitis and necrosis. As a consequence, there may be bleeding or ischemia of organs or tissues supplied with blood by diseased vessels (e.g., a stroke). What are the causes, symptoms, and types of systemic vasculitis? What is the treatment of this group of diseases?
Systemic vasculitis(vasculitis) is a group of systemic connective tissue diseases in the course of which the walls of the vessels become inflamed and necrotic. As a consequence, it may cause bleeding or the formation of blood clots and embolism, and further ischemia of tissues and organs supplied with blood by these vessels. This process can involve the vessels of various tissues and organs, but usually affects the kidneys, lungs, upper respiratory tract, skin, nervous system, and eyeballs.
The nervous system may develop mono- or polyneuropathy, or syndromes of peripheral nerve damage. On the part of the digestive system, for example, intestinal bleeding may appear. Liver damage can also occur.
The most dangerous consequence of systemic vasculitis is a stroke.
Disorders in the cardiovascular system, resulting from systemic vasculitis, include, among others: cardiomyopathies, pericarditis, valvular disease and stroke. In turn, in the respiratory system, systemic vasculitis can lead to alveolar bleeding, asthma or changes in the paranasal sinuses, and in the urinary system to glomerulonephritis. In addition, skin changes (hemorrhagic rash and ulcerations) or inflammation of joints and muscles may appear.
Systemic vasculitis - causes and risk factors
Vascular inflammation is believed to be initiated by immunological processes (associated with hypersensitivity to antigens). For example, some diseases belonging to the group of systemic small vasculitis are usually associated with the presence of antibodies to the neutrophil cytoplasm, abbreviated as ANCA.
The incidence of systemic vasculitis increases with age and mainly affects people over the age of 65.
Systemic vasculitis - types
There are several systemic classificationsvasculitis, however, currently the most commonly used classification was established at the Chapel Hill Conference (CHCC) and the American College of Rheumatology (ACR) classification:
1. Systemic small vasculitis (arterioles, venules and capillaries)
- granulomatosis with polyangiitis (Wegener's granulomatosis);
- microscopic polyangiitis;
- Churg-Strauss syndrome (eosinophilic granulomatous polyangiitis);
- IgA-related vasculitis (Henoch-Schönlein disease, allergic purpura);
- Goodpasture's syndrome (Goodpasture's disease);
- inflammation of the small vessels of the skin (skin leukocytoclastic vasculitis, hypersensitivity vasculitis);
- mixed cryoglobulinemia;
2. Systemic medium vasculitis (mainly visceral vessels, i.e. mesenteric, coronary and renal arteries, etc.)
- polyarteritis nodosa (aka Kussmaul disease, Kussmaul-Maier disease);
- Kawasaki disease, or mucocutaneous nodal syndrome;
3. Systemic large vasculitis (aorta and its largest branches),
- giant cell arteritis, aka Horton's syndrome;
- Takayasu's disease;
4. Systemic vasculitis of small, medium and large
- Behçet's disease
In addition, the following is used to divide systemic vasculitis into:
- primary - the cause of the disease is unknown;
- secondary - changes in the vessels occur in the course of other diseases (e.g. rheumatoid arthritis, systemic lupus, ulcerative colitis) or are caused by drugs;
Systemic vasculitis - treatment
Treatment of systemic vasculitis is primarily immunosuppressive therapy, including the administration of drugs such as glycosteroids and cyclophosphamide. In some cases, when the disease is very severe, plasmapheresis treatments (a method of cleansing the blood plasma of large particles such as immune complexes) are used.