The term "microscopic polyangiitis" refers to a serious disease, the course of which is accompanied by inflammation of small blood vessels, the formation of areas of necrosis in tissues that are located in close proximity to arterioles, venules and capillaries. The pathology belongs to the group of systemic vasculitis.
Currently, several methods have been developed for the treatment of microscopic polyangiitis. However, it is important to know that the disease is extremely difficult to treat. If conservative methods are ineffective, surgery is indicated.
General information
For the first time the disease was isolated in a separate nosological form in 1948. Scientists noted that during the development of polyarteritis nodosa, sooner or later, small blood vessels were affected, which led to the formationnecrotizing glomerulonephritis, but at the same time arterial hypertension was observed extremely rarely in patients. As a result, it was decided to separate this pathological condition into a separate nosology.
The expediency of this decision was due to the fact that microscopic polyangiitis is fundamentally different from polyarteritis nodosa. But at the same time, the disease combines the signs of Wegener's granulomatosis and rapidly progressive glomerulonephritis. The lungs, skin, and kidneys are most commonly affected.
According to ICD-10, microscopic polyangiitis is coded M31.7.
Reasons
The etiology of the disease is still unknown. Currently, tests are being carried out, based on the results of which it will be possible to assume what nature the pathology has - viral or immunogenetic.
Today, it is known that the pathogenesis of microscopic polyangiitis is directly related to the production of antibodies to the cytoplasm of neutrophils, which have a damaging effect on blood vessels. Medium and large arteries are rarely affected.
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 the lungs are involved in the pathological process, the disease is called hemorrhagic alveolitis. With lesions of the skin, it is customary to speak of leukocytoclastic venulitis.
The above pathologies have a number of symptoms, including specific ones. Clinical manifestations of microscopic polyangiitis include the following conditions:
- Low body temperature for a long time.
- Excessive sweating at night.
- Weakness, general malaise.
- Frequent episodes of arthralgia and myalgia.
- Hepatic and pulmonary syndromes.
- Disturbances in the work of the upper respiratory tract (sinusitis, rhinitis of atrophic or ulcerative necrotic nature, otitis media).
- Synovitis of articular joints (mainly interphalangeal and metacarpophalangeal).
- Irregularities in the work of the visual system. May manifest as keratitis, episcleritis, conjunctivitis, uveitis.
- Arthritis.
- Pain in the chest area.
- Hypertension.
- Respiratory failure.
- Shortness of breath that gets worse over time.
- Hemoptysis.
- Acute kidney failure.
- Intestinal or pulmonary bleeding.
- Presence of vascular purpura on the skin.
- Erythema.
- Soft tissue necrosis.
- Hematuria and proteinuria (the disease is one of the main causes of elevated C-reactive protein).
- Ischemic enterocolitis.
- Nephrotic and broncho-obstructive syndromes.
It is important to know that microscopic polyangiitis poses a threat not only to he alth, but also to the lives of patients. Most disturbingconditions are: hemoptysis, high degree of proteinuria, oliguria. The imminent onset of death is evidenced by: acute renal and respiratory failure, infectious complications, pulmonary bleeding.
Variants of the course of the disease
Medics divide microscopic polyangiitis into the following forms:
- Lightning fast. Characterized by the onset of death in a few weeks due to the development of acute renal failure or pulmonary hemorrhage.
- Sharp. Patients have glomerulonephritis, which progresses very quickly, or nephrotic syndrome.
- Recurrent. It is also called continuous. Exacerbation episodes occur about every 6-12 months. At the same time, non-specific clinical manifestations are observed in patients.
- Latent. In patients, predominantly articular syndrome, hemoptysis and hematuria are detected.
If you experience any alarming symptoms, you should consult a doctor. Microscopic polyangiitis is treated by an otorhinolaryngologist, phthisiatrician, ophthalmologist, pulmonologist, dermatologist, nephrologist, etc. The choice of a specialist directly depends on the affected area. If the presence of isolated syndromes is excluded, the pathology is treated by a rheumatologist.
Diagnosis
At the initial stage, the doctor will conduct an examination and take an anamnesis. After that, the specialist will prescribe a comprehensive examination. Diagnosticsmicroscopic 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 affected tissue. It can be a small piece of the lung, kidney, upper respiratory mucosa, skin.
- Scintigraphy.
- X-ray examination.
- CT.
- Ultrasound.
- Radioisotope research.
Based on the results of the diagnosis, the doctor will choose the tactics of treatment.
Drug therapy
All ongoing activities are aimed at preventing dysfunction and destruction of internal organs, achieving a period of stable remission, improving the quality and length of life.
According to clinical guidelines, microscopic polyangiitis is a disease whose treatment should be multi-stage. In this regard, the classical scheme of therapy consists of the following points:
- Achieve stable 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 to carry out therapeutic measures. Patients are scheduled to receive or administer cytostatics and Prednisolone.
- Phase cuppingexacerbations. Currently, the most effective method of treatment in case of relapse has not been developed. If the exacerbation is mild in nature, doctors prescribe the introduction of glucocorticosteroids in high dosages. 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.
Surgery
In some cases, the use of conservative methods does not lead to positive results. If, due to microscopic polyangiitis, patients develop end-stage renal failure, they are indicated for organ transplantation. To date, kidney transplantation is the only possible method of surgical treatment.
Forecast
In most cases, it is unfavorable. It is important for patients to know that they need very long-term therapy. In addition, they regularly (throughout their lives) need to come to a rheumatologist for a checkup.
According to statistics, the five-year survival rate with treatment is only 65%. It is customary to talk about a poor prognosis if the patient has hemoptysis. Old age is a risk factor.
The main causes of death: acute renal or respiratory failure, pulmonary hemorrhage, infectious complications.
In closing
Microscopic polyangiitis -is a disease related to systemic vasculitis. It is characterized by inflammation of small blood vessels. In addition, there is a death of tissues located in their immediate vicinity. The reasons for the development of pathology are still unknown.
It is important to know that the disease is a danger not only to he alth, but also to the life of the patient. In this regard, it is necessary to consult a doctor at the first warning signs.
