Systemic vasculitis - generalizing the generic namegroup of diseases in which a number of bodies or body tissues are involved in the disease process. At the heart of these diseases is inflammation of the blood vessel wall, which is reflected in the name: the word "vasculitis" comes from the Latin. vasculum ( «vessel", "vial") and the Greek. -itis (suffix indicating inflammation). Synonyms of the word "vasculitis" are less likely to use the term "vasculitis" (from the Greek angion -. Jar) and "arteritis".
Types of systemic vasculitis
From a practical point of view, important is the fact that the vasculitis are primary and secondary.
Secondary vasculitis develops as an elementother disease and can be treated as either optional (optional), its manifestation, or as a complication. Examples are many infectious diseases: scarlet fever, typhus, meningitis, sepsis - as well as skin conditions such as psoriasis, etc. Sometimes vasculitis may be one of the manifestations of cancer of an organ;. in this case the secondary vasculitis is also because, after successful surgery, radiotherapy or chemotherapy treatment of a tumor associated vasculitis it usually disappears without any special treatment.
Primary vasculitis are independentdiseases related primarily to the competence of rheumatologists, although diagnosis and treatment of these diseases is not possible without the participation of doctors of other specialties: otolaryngologists, ophthalmologists, neurologists, dermatologists, etc. Inflammation of the blood vessel wall - an essential feature of these diseases, which is found in all without exception cases. . Among the primary systemic vasculitis include nonspecific aortoarteriit (synonyms: "Takayasu's disease," "aortic arch syndrome"); giant cell arteritis (synonyms: "temporal arteritis", "senile arteritis", "Horton's Disease"); polyarteritis nodosa; Kawasaki disease; Wegener's granulomatosis; microscopic polyangiitis; eosinophilic angiitis and granulomatosis (synonym: "Chёrga-Strauss syndrome"); cryoglobulinemic vasculitis; Henoch-Schönlein purpura (synonym: "hemorrhagic vasculitis"). In all of these diseases, there is inflammation in the vessel wall, but the caliber of the affected blood vessels in various forms of vasculitis varies and ranges from large arteries (diameter of 1.0 cm or more) to the smallest arterioles, capillaries and venules, visible only under a microscope. Varies and the type of inflammation. All this explains the very marked diversity of clinical manifestations of primary systemic vasculitis and their difference from each other.
The causes and mechanism of development of systemic vasculitis
Fold the question about the causes of primarysystemic vasculitis. In the most general form of the mechanism of occurrence of vasculitis explain immune dysfunction of the immune system when the body's cells and they produce aggressive substances begin to attack its own tissues and organs. In turn, this may cause dysfunction of different factors.
Of great importance are infections - both acuteand chronic - as in recent years shows that some infectious agents are able to "deceive" the immune system, distort the immune response perenatselivaya it with microbial bodies in the internal structures of the body. At least at two systemic vasculitis attributable to the "primary" - polyarteritis nodosa and vasculitis cryoglobulinemic - proved the role of viruses, respectively, hepatitis B virus and hepatitis C virus Matter foci of chronic bacterial (purulent) infection, especially chronic tonsillitis ( recurrent angina).
Other risk factors that could potentiallycause the development of vasculitis include the abuse of drugs, the uncontrolled introduction of vaccines and serums, overreliance on "tan" and exposure to the sun, prolonged hypothermia. Sometimes the role of "trigger" perform physical trauma, a psychoemotional overstrain or alcohol (sometimes even small amounts of alcohol suddenly find themselves able to trigger the development of the disease).
Of course, the human body in normalconditions easily named resists all kinds of influences; therefore suggest that the vasculitis develop only as a result of the simultaneous combined effect of several factors, when possible adaptation of protective systems are exceeded.
The failure protection system may also beconnected with the peculiarities of the internal hormonal status: some vasculitis (eg, non-specific aortoarteriit Takayasu and giant cell arteritis) more likely to develop in women, due to predisposing to high blood levels of estrogen - the female sex hormones - causing an imbalance in the immune system function; At the same time, other vasculitis (such as polyarteritis nodosa), by contrast, often affect men.
The scientific explanation of all of these processes sufferMore numerous gaps and logical inconsistencies, much remains unclear and requires further study. Not completely clear, for example, the role of hereditary (genetic) predisposition to the development of systemic vasculitis: a few cases noted the occurrence of vasculitis in families; It identified some of the genes that occur with greater frequency among cases systemic vasculitis than among other individuals. It is shown that in case of need a kidney transplant patients with systemic vasculitis, which caused heavy damage to the kidneys, it is undesirable to use a donor organ obtained from a close relative, as the genetic relationship increases the risk of recurrence of vasculitis in the transplanted kidney. However, in most cases of vasculitis are not inherited from parents to children, and the risk of developing these diseases in the offspring is minimal.