The term "microscopic polyangiitis" refers to a serious illness, the course of which is accompanied by inflammation of small blood vessels, the formation of sites of necrosis in tissues that are located in close proximity to arterioles, venules and capillaries. Pathology belongs to the group of systemic vasculitis.
Currently, several methods for the treatment of microscopic polyangiitis have been developed. However, it is important to know that the disease is extremely difficult to treat. With the ineffectiveness of conservative methods, surgical intervention is indicated.
General information
The disease was first isolated in a separate nosological form in 1948. Scientists noted that, during the development of polyarteritis nodosa, small blood vessels were affected sooner or later, which led to the formation of necrotizing glomerulonephritis, but arterial hypertension was extremely rare in patients. As a result, it was decided to isolate this pathological condition into a separate nosology.
The feasibility of this solution was due to the fact that microscopic polyangiitis is fundamentally different from polyarteritis nodosa. But at the same time, the disease combines the symptoms of Wegener's granulomatosis and rapidly progressive glomerulonephritis. The most commonly affected are the lungs, skin, and kidneys.
According to ICD-10, code M31.7 is assigned to microscopic polyangiitis.
Causes
The etiology of the disease is still unknown. Tests are currently underway, based on the results of which it will be possible to assume what nature the pathology has - viral or immunogenetic.
Today, the fact is known that the pathogenesis of microscopic polyangiitis is directly related to the production of antibodies to the neutrophil cytoplasm, which have a damaging effect on blood vessels. Medium and large arteries are extremely rare.
Symptoms
Doctors call the disease differently (depending on which organ is involved in the pathological process). If we are talking about microscopic polyangiitis with kidney damage, it is customary to talk about necrotizing glomerulonephritis. When involved in the pathological process of the lungs, the disease is called hemorrhagic alveolitis. With damage to the skin, it is customary to talk about leukocytoclastic venulitis.
The above pathologies have a number of symptoms, including specific ones. The clinical manifestations of microscopic polyangiitis include the following conditions:
- Low body temperature for a long time.
- Excessive perspiration at night.
- Weakness, general malaise.
- Frequent episodes of arthralgia and myalgia.
- Hepatic and pulmonary syndromes.
- Disorders in the upper respiratory tract (sinusitis, rhinitis of atrophic or ulcerative necrotic nature, otitis media).
- Synovitis of articular joints (mainly interphalangeal and metacarpophalangeal).
- Violations of the visual system. May occur in the form of keratitis, episiscleritis, conjunctivitis, uveitis.
- Arthritis
- Pain in the chest.
- Arterial hypertension.
- Respiratory failure.
- Shortness of breath, the severity of which increases over time.
- Hemoptysis.
- Acute renal failure.
- Intestinal or pulmonary hemorrhage.
- The presence of vascular purpura on the skin.
- Erythema.
- Soft tissue necrosis.
- Hematuria and proteinuria (the disease is one of the main causes of increased C-reactive protein).
- Ischemic enterocolitis.
- Nephrotic and bronchial obstructive syndromes.
It is important to know that microscopic polyangiitis is a threat not only to health, but also to the lives of patients. The most alarming conditions are: hemoptysis, a high degree of proteinuria, oliguria. The imminent death is indicated by acute renal and respiratory failure, complications of an infectious nature, pulmonary hemorrhage.
Variants of the course of the disease
Doctors divide microscopic polyangiitis into the following forms:
- Lightning fast. It is characterized by the onset of death in a few weeks due to the development of acute renal failure or pulmonary hemorrhage.
- Subacute In patients, glomerulonephritis is detected, which progresses very quickly, or nephrotic syndrome.
- Recurrent. It is also called continuous. Exacerbation episodes occur approximately every 6-12 months. Moreover, non-specific clinical manifestations are observed in patients.
- Latent. In patients, mainly articular syndrome, hemoptysis and hematuria are detected.
If any alarming symptoms occur, consult a doctor. Otolaryngologist, TB specialist, ophthalmologist, pulmonologist, dermatologist, nephrologist, etc. are involved in the treatment of microscopic polyangiitis. The choice of a specialist directly depends on the affected area. If the presence of isolated syndromes is excluded, a rheumatologist is involved in the treatment of the pathology.
Diagnostics
At the initial stage, the doctor will conduct an examination and collect an anamnesis. After that, the specialist will prescribe a comprehensive examination. Diagnosis of microscopic polyangiitis includes:
- General analysis of urine and blood.
- Test for creatinine, electrolytes, fibrinogen and C-reactive protein. The reason for their increased values is often polyangiitis.
- Analysis to determine the concentration of iron in the blood.
- Biopsy of the affected tissue. This may be a small piece of the lung, kidney, mucosa of the upper respiratory tract, skin.
- Scintigraphy.
- X-ray examination.
- CT
- Ultrasound
- Radioisotope study.
Based on the results of the diagnosis, the doctor will choose the treatment tactics.
Drug therapy
All measures taken are aimed at preventing dysfunction and destruction of internal organs, achieving a period of stable remission, and improving the quality and longevity of life.
According to clinical recommendations, microscopic polyangiitis is a disease whose treatment should be multi-stage. In this regard, the classical therapy regimen consists of the following items:
- Achieving persistent remission. The pathological process in the active phase requires the introduction of drugs related to cyclophosphamides and glucocorticosteroids. In some cases, plasmapheresis is prescribed to increase the degree of effectiveness.
- Maintaining stable remission. Even with the onset of this period, it is necessary to continue therapeutic measures. Patients are prescribed or administered cytostatics and Prednisolone.
- Relief of the phase of exacerbation. Currently, the most effective treatment for relapse has not been developed. If the exacerbation is mild, the doctors prescribe the introduction of glucocorticosteroids in high doses. If the relapse is serious, measures are taken to achieve a period of stable remission.
If necessary, the attending physician can make adjustments to this scheme.
Surgical intervention
In some cases, the use of conservative methods does not lead to positive results. If, due to microscopic polyangiitis, patients develop terminal renal failure, they are shown organ transplantation. Today, kidney transplantation is the only possible surgical treatment.
Forecast
In most cases, it is unfavorable. It is important for patients to know that they need a very long therapy. In addition, they regularly (throughout life) need to visit a rheumatologist for an examination.
According to statistics, five-year survival during treatment is only 65%. It is customary to talk about a poor prognosis if the patient has hemoptysis. A risk factor is old age.
The main causes of death: acute renal or respiratory failure, pulmonary hemorrhage, complications of an infectious nature.
Finally
Microscopic polyangiitis is a disease related to systemic vasculitis. It is characterized by inflammation of small blood vessels. In addition, tissue that is in their immediate vicinity dies. The reasons for the development of pathology are still unknown.
It is important to know that the ailment is a danger not only to health, but also to the life of the patient. In this regard, the doctor must be consulted when the first alarming signs appear.