Idiopathic fibrosing alveolitis: treatment
Last reviewed: 23.04.2024
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Idiopathic fibrosing alveolitis (Hammen-Rich disease) is a pathological process in the alveoli and incruscative lung tissue of an unclear nature, leading to progressive fibrosis and accompanied by an increasing respiratory insufficiency.
The cause of the development of idiopathic fibrosing alveolitis is unknown. The pathogenesis of the disease is not fully understood. It is assumed that interstitial lung tissue decreases collagen degradation and increases its synthesis by fibroblasts and alveolar macrophages. The increase in the synthesis of collagen is facilitated by an increase in the number of individual subpopulations of lymphocytes that react to the collagen of the lung tissue as a foreign protein and produce lymphokines that stimulate the formation of collagen. Simultaneously, the production of lymphocytes "inhibiting factor", which normally inhibits the synthesis of collagen, decreases.
Many people consider this disease autoimmune. Antigen-antibody complexes are deposited in the walls of small vessels of the lungs. Under the influence of circulating immune complexes, lysosomal enzymes of alveolar macrophages and neutrophils, the lung tissue deteriorates, the thickening of the interalveolar septa, the obliteration of the alveoli and capillaries by fibrous tissue.
The main drugs for the treatment of fibrosing alveolitis are glucocorticoids and D-penicillamine. These drugs are prescribed taking into account the phase of the disease.
According to Μ. Μ. Ilkovich (1983), in the stage of interstitial edema and alveolitis (this stage is diagnosed clinically and radiological), glucocorticoids are prescribed. They cause anti-inflammatory and immunosuppressive effect, inhibit the development of fibrosis.
Patients with idiopathic fibrosing alveolitis with early diagnosis (phase of edema and alveolitis phase) are prescribed 40-50 mg of prednisolone for 3-10 days, then gradually (depending on the effect) dose is reduced for 6-8 months to maintain (2.5-5 mg per day). The duration of treatment is an average of 18-20 months.
When the pathological process passes to the stage of interstitial fibrosis, the appointment of D-penicillamine in combination with prednisone is indicated, the initial dose of which in this case is 15-20 mg per day.
It was established that in patients with fibrosing alveolitis in the blood serum the content of copper is increased, which contributes to the collagenization of the interstitial lung stroma. D-penicillamine inhibits copper-containing aminoxidase, which reduces the copper content in the blood and lungs and inhibits the maturation and synthesis of collagen. In addition, D-penicillamine has an immunosuppressant effect.
MM Il'kovich and LN Novikova (1986) suggest that in the chronic course of the disease without pronounced exacerbations, prescribe D-penicillamine 0.3 g per day for 4-6 months, then 0.15 g / day for 1-1.5 of the year.
In the acute course and exacerbation of the disease, they suggest that D-penicillamine should be prescribed 0.3 g per day in the first week, 0.6 g per day in the second, 1.2 g per day in the third, and then reduced in the reverse order. The maintenance dose is 0.15-0.3 g per day for 1-2 years.
With pronounced changes in the immunological status, azathioprine is prescribed (has a pronounced immunosuppressive effect) according to the following scheme: 150 mg per day for 1-2 months, then 100 mg per day for 2-3 months, then maintain a dose (50 mg per day) for 3-6 months. The average duration of azathioprine is 1.9 years.
The effectiveness of immunosuppressive agents is enhanced by the use of hemosorption, which facilitates the removal of circulating immune complexes.
In the treatment of idiopathic fibrosing alveolitis, it is advisable to use aldactactone (veroshpirona), it reduces alveolar and interstitial swelling, has an immunosuppressive effect. The daily dose of veroshpiron is 25-75 mg, the duration of treatment is 10-12 months.
In addition, antioxidants are recommended (vitamin E - 0.2-0.6 g of 50% solution per day).
In the early stages of the disease, sodium thiosulfate is used intravenously (5-10 ml of 30% solution) for 10-14 days. The drug has antioxidant, antitoxic, anti-inflammatory and desensitizing effects.
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