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Idiopathic fibrosing alveolitis - Treatment
Last reviewed: 04.07.2025
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Idiopathic fibrosing alveolitis (Hamman-Rich disease) is a pathological process in the alveoli and incremental tissue of the lungs of unclear origin, leading to progressive fibrosis and accompanied by increasing respiratory failure.
The cause of idiopathic fibrosing alveolitis is unknown. The pathogenesis of the disease has not been fully elucidated. It is assumed that collagen breakdown in the interstitial tissue of the lungs decreases and its synthesis by fibroblasts and alveolar macrophages increases. Increased collagen synthesis is facilitated by an increase in the number of individual lymphocyte subpopulations that react to pulmonary collagen as a foreign protein and produce lymphokines that stimulate collagen formation. At the same time, the production of "inhibitory factor" by lymphocytes, which inhibits collagen synthesis under normal conditions, decreases.
Many consider this disease to be 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, damage to the lung tissue, compaction, thickening of the interalveolar septa, obliteration of the alveoli and capillaries by fibrous tissue occurs.
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 M. M. Ilkovich (1983), glucocorticoids are prescribed at the stage of interstitial edema and alveolitis (this stage is diagnosed clinically and radiologically). They cause an anti-inflammatory and immunosuppressant effect, inhibit the development of fibrosis.
Patients with idiopathic fibrosing alveolitis, if the diagnosis is made early (edema phase and alveolitis phase), are prescribed 40-50 mg of prednisolone for 3-10 days, then gradually (depending on the effect) the dose is reduced over 6-8 months to a maintenance dose (2.5-5 mg per day). The duration of treatment is on average 18-20 months.
When the pathological process progresses to the stage of interstitial fibrosis, the administration of D-penicillamine in combination with prednisolone is indicated, the initial dose of which in this case is 15-20 mg per day.
It has been established that patients with fibrosing alveolitis have elevated copper levels in their blood serum, which promotes collagenization of the interstitial stroma of the lungs. D-penicillamine inhibits copper-containing aminooxydase, which reduces copper levels in the blood and lungs and inhibits collagen maturation and synthesis. In addition, D-penicillamine has an immunosuppressant effect.
M. M. Ilkovich and L. N. Novikova (1986) suggest prescribing D-penicillamine at 0.3 g per day for 4-6 months for chronic disease without clearly defined exacerbations, then 0.15 g per day for 1-1.5 years.
In acute cases and exacerbations of the disease, they suggest prescribing D-penicillamine at 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 reducing the dose in reverse order. The maintenance dose is 0.15-0.3 g per day for 1-2 years.
In case of significant 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 a maintenance dose (50 mg per day) for 3-6 months. The average duration of azathioprine intake is 1.9 years.
The effectiveness of immunosuppressant agents is increased by the use of hemosorption, which promotes the removal of circulating immune complexes.
In the treatment of idiopathic fibrosing alveolitis, it is advisable to use aldactone (veroshpiron), it reduces alveolar and interstitial edema, 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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