Idiopathic fibrosing alveolitis in children

Idiopathic fibrosing alveolitis (ICD-10 code: J84.1) refers to interstitial diseases of the lungs of unknown etiology. In medical literature, synonyms are used: Hammam-Rich's disease, acute fibrosing pulmonitis. Fibrotic dysplasia of the lungs. In children, idiopathic fibrosing alveolitis is rare.

[1], [2], [3], [4]

What causes idiopathic fibrosing alveolitis in children?

A possible genetic predisposition is indicated by family cases of the disease. The pathogenesis of idiopathic fibrosing alveolitis is caused by pulmonary fibrosis, the deposition of immune complexes in the capillaries of the alveoli, and the influence of organ-specific antibodies. The pathological process is localized in the alveolar interstitium, which leads to its diffuse fibrosis.

Symptoms of idiopathic fibrosing alveolitis

The acute onset of the disease is characterized by febrile temperature, dyspnea, dry cough. The onset of the disease is often subacute - there are complaints of increased fatigue, a rare cough, shortness of breath only with physical exertion. As the disease progresses, dyspnea increases, the chest becomes flattened, its excursion and circumference decreases, the amplitude of respiratory movements decreases, the body weight and height fall behind the norm, deformations of the distal phalanges of the fingers appear in the form of "watch glasses" and "drum sticks", acrocyanosis and cyanosis of the nasolabial triangle acquire a permanent character. At auscultation, scattered small bubbling wet or crepitic rales are heard.

Idiopathic fibrosing alveolitis has a steadily progressing course. Pressure in the pulmonary artery increases with the formation of a chronic pulmonary heart and right ventricular failure. Often, with idiopathic fibrosing alveolitis, complications develop in the form of arthritic syndrome, spontaneous pneumothorax, pleural exudates, and rarely pulmonary embolism.

Diagnosis of idiopathic fibrosing alveolitis

[5], [6], [7], [8], [9]

Laboratory diagnosis of idiopathic fibrosing alveolitis

Characteristic for idiopathic fibrosing alveolitis are changes in the immunogram: increased IgG (up to 2000-4000 mg / l) and circulating immune complexes (up to 150 UE). There is also an increase in ESR.

Instrumental diagnosis of idiopathic fibrosing alveolitis

At an early stage of the disease, an x-ray picture of the chest may not be affected. Subsequently, a decrease in the transparency of the lung tissue (a symptom of "frosted glass"), a reticular-drawing pattern, shallow focal shadows is revealed. In the process of progressing fibrosis appear tight compaction, cellular enlightenment, pulmonary fields are lent, stipulating a high standing of the diaphragm domes, widening of the intrathoracic part of the trachea and large bronchi, expansion of the arc and branches of the pulmonary artery, a honeycomb honeycomb is formed.

When carrying out scintillas of lungs there is a local decrease in pulmonary blood flow to 60-80%. The degree of fibrosis development can be judged from the FVD data: a restrictive type of ventilation disorders, a decrease in the diffusivity of the lungs, a decrease in the extensibility of the pulmonary tissue, and hypoxemia.

Holding bronchography is not informative.

Bronchoscopy in the cytology of the bronchoalveolar fluid is dominated by neutrophils and lymphocytes. Transbronchial biopsy is informative only in half the cases. An open lung biopsy can confirm the diagnosis of idiopathic fibrosing alveolitis in 90% of cases.

Treatment of idiopathic fibrosing alveolitis

Treatment of idiopathic fibrosing alveolitis is long, complex, with constant correction depending on the course of the disease. Glucocorticoids are combined with immunosuppressants. Hormonal drugs are especially effective in the early stages, when the alveolitis predominates over the processes of fibrosing of the lung tissue.

Prednisolone is prescribed in a dose of 1-1.5 mg / kg per day for 3-6 weeks. The dose is reduced gradually (2.5-5 mg per week). A maintenance dose of 2.5-5 mg / day is taken for 9-12 months. With a rather severe condition and rapid progression of fibrosis, therapy can begin with intravenous administration of glucocorticoids at the maximum dose, followed by a transition to oral or pulse therapy. In addition to glucocorticoids, D-penicillamine (coulenyl) is administered at a dose of 125-250 mg / day for 8-12 months (dose-related doses). Basic therapy courses are repeated periodically.

Treatment of right ventricular failure is carried out according to the traditional scheme in parallel.

It is important to note that exacerbations of the disease are usually provoked by the attachment of SARS, which are difficult to differentiate from bacterial complications, therefore, antibiotics are additionally prescribed. In severe respiratory distress (for p _and O ₂ less than 60 mm Hg. V.) Is assigned to a long course of oxygen therapy. Showed a light chest massage, special breathing exercises, exercise therapy, plasmapheresis and lymphocytosis. Vitamins and potassium preparations are added.

What prognosis does idiopathic fibrosing alveolitis have in children?

Idiopathic fibrosing alveolitis in children has an unfavorable prognosis, depends on the timely diagnosis and the beginning of adequate therapy.