System vaskulita unite group of diseases at which the inflammation and destruction of vascular walls leading to ischemia of bodies and fabrics is observed. System vaskulita are presented by Takayasu's arteritis, granulematozy Wegener, a nodular periarteriit, Bekhchet's syndrome obliterating trombangiity, gigantokletochny arteritis, etc. System vaskulita are burdened by damage of skin, kidneys, hearts, lungs, joints, organs of vision etc. The diagnosis is confirmed with laboratory tests, results of a biopsy and visceral angiography. Therapy by glucocorticoids, cytostatics, vascular medicines allows to reduce damage of vitals, to achieve clinical remission.
The diseases presented by primary system vaskulita develop independently and are characterized by a nonspecific inflammation of vascular walls. Emergence of primary system vaskulit is connected with violation of the immune responsiveness caused, as a rule, by the infectious agent. The inflammation at system vaskulita mentions all layers of a vascular wall: it can have destructive character, cause vessel occlusion, microcirculator frustration and, as a result, ischemia of bodies up to necroses and heart attacks.
Secondary vaskulit is an element of other pathology and is considered as its local facultative manifestation or a complication (for example, vaskulit at scarlet fever, meningitis, a typhus, sepsis, psoriasis, tumors etc.). More often men of average age group suffer from system vaskulita.
Classification of system vaskulit
Distinguish system vaskulita with defeat of vessels of small, average and large caliber. The group of vascular defeats of small vessels is presented granulematozy Wegener, Chardzh-Stross's syndrome, a microscopic poliangiit, an essentsialny krioglobulinemichesky vaskulit, purpury Shenleyna-Genokh. System vaskulita with interest of vessels of average caliber include a disease of Kawasaki and nodular periarteriit (polyarteritis). Takayasu's disease and arteritis gigantokletochny temporal belong to system vaskulita of large vessels.
Symptoms of system vaskulit
The clinic of system vaskulit includes various general nonspecific symptoms: fever, appetite loss, astenisation, weight loss. The skin syndrome at system vaskulita is characterized by hemorrhagic rash, ulcerations, skin necroses. Musculoarticulate defeats are shown by mialgiya, artralgiya, arthritises. Changes from peripheral nervous system at system vaskulita proceed in the form of a polyneuropathy or multiple mononeuropathy. The clinic of visceral defeats can be shown by strokes, a myocardial infarction, damage of eyes, kidneys, lungs etc.
At a nonspecific aortoarteriit (Takayasu's disease) the aorta arch with the branches departing from it is involved in a granulematozny inflammation. The disease is shown by generalized pain, microcirculator violations in the top extremities, involvement of vessels of a bryzheyka and lungs, stenocardia, heart failure. Arteritis gigantokletochny temporal (Horton's disease), entering into group of system vaskulit, proceeds with an inflammation sleepy, temporal, is more rare than vertebral arteries. The clinic of damage of arteries is shown by headaches, a giperesteziya and puffiness of skin of temporal area, eye symptomatology, neurologic violations.
The disease of Kawasaki proceeding with a mucous and skin and ferruterous syndrome children are surprised. The clinic includes fever, conjunctivitis, diffusion spotty and papular rash, erity, hypostases, a skin peeling. At this form of a system vaskulit the limfadenopatiya, defeat of language, mucous lips is noted. When involving coronary arteries aneurisms, heart attacks can develop.
Nekrotiziruyushchy changes in arteries of average and small caliber and signs of polysystem defeats are characteristic of nodular pan-arteritis (polyarteritis, a periarteriit). Against the background of a general malaise a kidney syndrome (glomerulonefrit, malignant hypertensia, a renal failure), artralgiya, polyneuritis, a pneumonitis develop, koronarit, microstrokes.
Shenleyn-Genokh's disease belongs to the system vaskulita striking small vessels as a result of the postponed pharyngitis of a streptococcal etiology. Arises usually at children; proceeds with melkopyatnisty hemorrhages, poliartralgiya and polyarthritis, an abdominal syndrome, immunocomplex glomerulonefrit.
Angiit allergic or the syndrome of Cherdzha-Stross is connected with eozinofilny infiltration of walls of vessels; proceeds with clinic of bronchial asthma and eozinofilny pneumonia, a polyneuropathy. Usually develops at the persons with the allergic anamnesis and also who visited the tropical countries. At a system granulematozny vaskulit of Wegener vessels of a pneumatic path and kidneys are surprised. The disease is shown by development of sinusitis, ulcer and necrotic rhinitis, cough, breath difficulty, a blood spitting, nephrite symptoms.
The syndrome of a giperergichesky system vaskulit (skin or leykotsitoklastichesky vaskulit) proceeds with an immunocomplex inflammation of capillaries, and faded. Skin manifestations (purple, blisters, ulcerations) and polyarthritis are typical. Development of nekrotiziruyushchy arteritis, a glomerulonefrit and kapillyarit of the pulmonary course is specific to a microscopic poliangiit.
System vaskulit, proceeding in the form of an essentsialny krioglobulinemiya, is caused by an inflammation and occlusion of the vessels caused by influence of krioglobulinovy complexes. The reciprocal simptomokompleks develops after treatment by penicillin, sulfanylamides, etc. drugs; it is shown purpury, urticaria, arthritises, artralgiya, a limfadenopatiya, glomerulonefrity. It is inclined to regression of manifestations after an etiofaktor exception.
Diagnostics of system vaskulit
During diagnostics of a system vaskulit comprehensive examination of the patient with consultation of the rheumatologist, neurologist, nephrologist and other experts is conducted. Carry out the ECG, an echocardiography, ultrasonography of kidneys, a X-ray analysis of lungs, etc. Growth of SOE serves as a characteristic, but nonspecific sign of system vaskulit. In blue blood At to cytoplasm of neutrophils (ANCA) and the CEC are defined.
At an angiographic research comes to light vaskulit vessels of small and average diameter. The greatest diagnostic importance at system vaskulita the biopsy of the struck fabrics and their subsequent morphological studying has.
Treatment of system vaskulit
The main stages of therapy of system vaskulit consist of suppression of the hyperimmune answer for the purpose of induction of clinical laboratory remission; carrying out an immunosupressorny course; maintenance of permanent remission, correction of organ violations and rehabilitation. Carrying out an anti-inflammatory and immunosupressivny course kortikosteroidny hormones is the cornerstone of pharmacotherapy of system vaskulit (Prednisolonum, dexamethasone, triamtsinolony, betametazony). Carrying out pulse therapy methylprednisolonum is effective.
Medicines of tsistostatichesky action at system vaskulita are applied in cases of generalization and progressing of process, malignant renal hypertensia, defeats of TsNS, inefficiency of kortikosteroidny therapy. Application of tsitostatik (a tsiklofosfan, a methotrexate, an azatioprin) allows to achieve suppression of immune mechanisms of an inflammation. Effectively and quickly the biological medicines inactivating FNO eliminate an inflammation (, ); NPVS (ibuprofen, , diclofenac).
Therapy by anticoagulants and antiagregant (heparin, dipiridamoly, pentoksifilliny) is shown at signs of hyper coagulation and the DVS-syndrome. Violations of peripheral microcirculation korrigirutsya by purpose of nicotinic acid and its derivatives. In therapy of system vaskulit vasoprotectives, vazodilatator, blockers of calcic channels are used.
Except medicamentous courses at system vaskulita holding sessions of the extracorporal haemo correction (a krioaferez and a cascade filtration of plasma) allowing to remove the circulating antibodies and immune complexes from blood is shown. Therapy of the complicated system vaskulit demands the coordinated cooperation of the rheumatologist, the nephrologist, the pulmonologist, the otolaryngologist, the neurologist, the surgeon, the oculist, etc.
Forecast and prevention of system vaskulit
The prospect of health and working capacity at system vaskulita is defined by a pathology form, age of the diseased, efficiency of treatment. Complex and landmark treatment of system vaskulit reduces number of lethal and invalidiziruyushchy outcomes.
Prevention of system vaskulit consists in an exception of contacts with infectious agents and allergens, refusal of unreasonable inoculations and prescription of medicines.