Systemic vasculitis symptoms
Vasculitis is a group of diseases in which the pathogenesis of which is caused by inflammation of the vascular wall. When the disease affects several tissues and organs: blood supply to the organ is impaired, blood vessels contract, which leads to tissue death.
Symptoms of
When skin is affected with vasculitis, a characteristic rash occurs. If nerves were damaged, then to a certain extent, the sensitivity of a person is broken, starting from hypersensitivity and ending with its complete loss. Because of renal vasculitis, renal failure may occur. Among the common symptoms of vasculitis can be noted weakness and fatigue, loss of appetite, pallor, increased body temperature and more.
If you constantly see abdominal pain, mental disorders, muscle pain, weight loss, then these are the typical symptoms for nodular vasculitis. Giant cell arteritis can cause symptoms such as very severe headaches, fever, permanent weakness and weight loss, swelling in the temples and some other symptoms.
Inflammations differ in the morphological pattern, common for them is the infiltration of the vessel wall by blood cells in the form of clusters or granules. Tissue changes are manifested by swelling, proliferation of the endothelium, thrombosis, hemorrhages, necrosis. In connection with this, the diseases of this group are called necrotizing vasculitis.
Clinical manifestations of systemic vasculitis are difficult to identify and verify in the form of clearly defined syndromes, as transitional forms are often observed or they develop in the framework of other diseases( vasculitis in tumors, intestinal and liver pathologies, after transplantation, etc.).
Groups
In terms of the role of immunopathological mechanisms, systemic vasculitis is divided into three main groups.
Vasculitis associated with immune complexes.
Hemorrhagic vasculitis( purpura Schönlein-Genocha);
Cryoglobulinemic vasculitis.
Vasculitis with SLE and rheumatoid arthritis.
Serum sickness.
Infectious vasculitis.
Paraneoplastic vasculitis.
Behcet's disease.
Vasculitis associated with organ-specific AT.
Goodpasture syndrome( AT to the basal membrane of the glomeruli of the kidney).
Kawasaki disease( AT to endothelium).
Vasculitis associated with AT to the cytoplasm of neutrophils:
Wegener's granulomatosis.
Microscopic polyarteritis( polyangiitis).
Syndrome Cherdja Strauss.
Classic nodular polyarteritis( rarely).
Some medicinal vasculitides.
Forms of
There are two forms of vasculitis: primary and secondary.
Primary vasculitis acts as an independent disease, the cause of which has not been clarified to date. Secondary vasculitis means damage to the walls of blood vessels in various diseases.
Secondary vasculitis may be a symptom of:
acute and chronic diseases of infectious genesis;
the genetic predisposition of a particular person to the occurrence of vasculitis;
individual response to vaccination;
contact with biological poisons, which are part of some drugs, for example, used for cancer;
effects on serum or chemicals;
strong body overheating;
prolonged exposure to low temperature;
injuries of different localization and origin;
thermal damage to the skin, in particular, with sunburn.
The role and importance of immune complexes in the development of systemic vasculitis in SLE and rheumatoid arthritis are described above.
Vasculitis of the second group is closely associated with certain types of AT.AT to the basal membrane of the glomerulus of the kidney have a high( more than 90%) diagnostic sensitivity and specificity for the Goodpasture syndrome. In recent years, evidence of the connection of Kawasaki's disease with AT to the endothelium has been obtained.
AT to the cytoplasm of neutrophils play an important role in the development of vasculites that are part of the third group. They are a complex of AT to various granulocyte, monocytic and, possibly, endothelial cytoplasmic Ar.
According to the fluorescence pattern in the determination of AT to the cytoplasm of neutrophils, they are divided into two types:
1) AT with classical diffuse fluorescence directed against protein kinase-3( they account for 85-90% of all ATs of this group) and a protein that enhances bactericidal actionneutrophils;
2) AT with perinuclear fluorescence is directed against myeloperoxidase( anti-MP, accounting for 90% of all ATs of this group), elastase, lactoferrin, cathepsin G and other polypeptides.
If the diagnosis of autoimmune liver diseases( especially primary sclerosing cholangitis) was sufficient to determine the total AT of the second type, then the entire spectrum of ATs to the cytoplasm of neutrophils is examined for the diagnosis of systemic vasculitis and other rheumatic diseases. For their detection, both the indirect immunofluorescence method and ELISA can be used. First, a screening test is recommended to identify total ANCA in the patient's serum, and then, if a positive result is obtained, use a test system for a particular individual AR.
Treatment of
Almost all vasculitis is a chronic progressive disease, when, periods of exacerbation are replaced by remission, which is achieved only by targeted treatment. At present, immunosuppression( immunosuppression) and anti-inflammatory treatment are given as the main directions of vasculitis treatment, which is provided by the joint application of glucocorticoids and / or cytotoxic drugs, which allow limiting or interrupting the development of immune inflammation.
The drug of choice is prednisolone, since it is able to quickly remove the swelling of the vascular wall, which leads to a decrease in the manifestations of ischemic syndrome. At the same time, prolonged use of glucocorticosteroids in a maintenance dose prevents further stenosis of large vessels.
However, it is believed that a significant improvement in the prognosis for life is associated with the use of cytotoxic drugs. Cyclophosphamide - the dose of this drug can vary within fairly wide limits, usually the initial dose in the period of pronounced clinical manifestations of the disease is 2 - 3 mg / kg per day. To intensify the therapy of vasculitis in severe torpid flow, combinations of high doses of methylprednisolone and cyclophosphamide at a dose of 1000 mg intravenously are used.
A good effect in treatment, as concomitant therapy, is vasodilator drugs( nicotinic acid, persantine, compliance) and antithrombotic drugs( quarantil, trental, agapurin) drugs are prescribed courses for a long time.
In recent years extracorporeal hemocorrection methods have been widely used to improve microcirculatory disorders( plasmapheresis in combination with hemoxygenation and ultraviolet irradiation of blood).Also with the purpose of correction of microcirculatory disorders, taking into account the leading mechanism that caused them, the following groups of drugs can be used: anticoagulants( direct and indirect) - heparin, fractiparin;methylxanthines - trental, agapurine;group of nonsteroidal anti-inflammatory drugs( small doses of aspirin - 75 - 125 mg / kg).
Carrying out active pathogenetic therapy throughout the disease allows not only to prolong the life of patients, but also to return some of them to work. This pathology can not be treated with herbal preparations, only those drugs listed here have a proven, positive effect.
Prevention
Given the causes of the disease, as a prophylaxis, you can recommend the following:
- hardening of the body;
- elimination of the negative impact of environmental pollution;
- exclusion of unreasonable use of medicines and administration of vaccinations.