Idiopathic fibrosing alveolitis: symptoms

Idiopathic fibrosing alveolitis develops most often between the ages of 40 and 70, with men 1.7-1.9 times more likely than women.

The most typical is a gradual, subtle start, but in 20% of patients the disease begins acutely with an increase in body temperature and pronounced dyspnea, but later the body temperature becomes normal or becomes subfebrile.

For idiopathic fibrosing alveolitis, the complaints of patients are extremely typical, and a careful analysis of which allows one to suspect this disease:

• shortness of breath is the main and permanent manifestation of the disease. At first, shortness of breath is less pronounced, but as the disease progresses, it grows and becomes so pronounced that the patient can not walk, serve himself, and even talk. The more severe and prolonged the disease, the more pronounced dyspnea. Patients note the constant nature of dyspnea, the absence of attacks of suffocation, but often emphasize the inability to take a deep breath. Because of progressive dyspnoea, patients gradually decrease their activity and prefer a passive lifestyle;
• cough is the second characteristic sign of the disease, about 90% of patients complain of coughing, but this is not the first symptom, it appears later, usually in the period of a pronounced clinical picture of idiopathic fibrosing alveolitis. Most often, cough is dry, but in 10% of patients it is accompanied by separation of mucous sputum;
• pain in the chest - are observed in half of patients, they are localized most often in the epigastric region on both sides and, as a rule, strengthen with deep inspiration;
• weight loss is a characteristic feature of idiopathic fibrosing alveolitis and worries patients usually in the phase of progressing disease; the degree of weight loss depends on the severity and duration of the pathological process in the lungs, possibly weight loss of 10-12 kg for 4-5 months;
• general weakness, fast fatigue, decreased performance - complaints characteristic of all patients, are especially pronounced in the progressing course of the disease;
• pain in the joints and morning stiffness in them - infrequent complaints, but can be markedly expressed in severe disease;
• an increase in body temperature is an uncharacteristic complaint for idiopathic fibrosing alveolitis, however, according to MM Ilkovichi, LN Novikova (1998), one-third of patients have subfebrile or febrile body temperature, most often between 10 and 13 hours . A fever indicates an active pathological process in the lungs.

Objective research reveals the following characteristic manifestations of idiopathic fibrosing alveolitis:

• dyspnoea and cyanosis of the skin and visible mucous membranes - initially observed mainly during physical exertion, and as the disease progresses, they become much stronger and become permanent; these symptoms are early signs in the acute form of idiopathic fibrosing alveolitis; with severe respiratory failure, a diffuse cyanosis of gray-ash color appears; the distinctive feature of respiration is the shortening of the phase of inhalation and exhalation;
• changes in nail phalanges (thickening of nail phalanges in the form of "drumsticks" and nails - in the form of "watch glasses" - Hippocratic fingers) - occur in 40-72% of patients, more often in men than women. This symptom is more natural with a pronounced activity and a long duration of the disease;
• changes in sound with percussion of the lungs - characterized by blunting over the area of the lesion, mainly in the lower parts of the lungs;
• characteristic auscultatory phenomena - weakening of vesicular breathing and crepitation. Weakening of vesicular breathing is accompanied by a shortening of the phases of inspiration and expiration. Creption is the most important symptom of idiopathic fibrosing alveolitis. It is heard from both sides, mainly on the back and middle axillary lines in the interblade area, in some patients (in the most severe course of the disease) - over the entire surface of the lungs. The crepitation resembles the "crackling of tselofan". In comparison with crepitation in other lung diseases (pneumonia, congestion), crepitus in idiopathic fibrosing alveolitis is more "tender", higher in frequency, less loud, is best heard at the end of the inspiration. As the pathological process progresses in the lungs, the "gentle" timbre of crepitation can be replaced by a more sonorous and coarse tone.

With a far-gone idiopathic fibrosing alveolitis, there is one more characteristic feature - "blotting out", which resembles in character a sound of friction or turn of the plug. The phenomenon of "peeing" is listened to by inhalation and mainly over the upper pulmonary fields and, mainly, in pronounced pleuropneumosclerotic processes.

In 5% of patients dry rales can be heard (usually with the development of concomitant bronchitis).

The course of idiopathic fibrosing alveolitis

ELISA progresses steadily and inevitably leads to the development of severe respiratory failure (manifested by severe shortness of breath, diffuse gray cyanosis of the skin and visible mucous membranes) and chronic pulmonary heart (compensated, then decompensated). The acute course of the disease is observed in 15% of cases and is manifested by severe weakness, shortness of breath, and high body temperature. In other patients, the onset of the disease is gradual, the course is slowly progressing.

The main complications of idiopathic fibrosing alveolitis are chronic pulmonary heart, severe respiratory failure with development of hypoxemic coma in the final. Less common are pneumothorax (with a formed "honeycomb lung"), thromboembolism of the pulmonary artery, exudative pleurisy.

Since the diagnosis of idiopathic fibrosing alveolitis patients live about 3-5 years. The main causes of death are severe heart and respiratory failure, PE, associated pneumonia, lung cancer. The risk of developing lung cancer in patients with EIA is 14 times higher than in the general population of the same age, sex, duration of smoking. Along with this, we report a possible recovery in such forms of idiopathic fibrosing alveolitis as desquamative interstitial, acute interstitial and nonspecific interstitial pneumonia with the use of modern methods of therapy.

AE Kogan, BM Kornev, Yu. A. Salov (1995) distinguish the early and late stages of idiopathic fibrosing alveolitis.

The early stage is characterized by respiratory insufficiency of the 1 st degree, an indistinctly pronounced immuno-inflammatory reaction, diffuse enhancement of the interstitial pattern without pronounced deformity. Patients complain of sweating, arthralgai, weakness. There is no cyanosis yet. In the lungs, "gentle" crepitation is heard, there is no hypertrophic osteoarthropathy (symptom of "drumsticks" and "watch glasses"). Examination and exudative-proliferative processes in interstitium predominate in the biopsy specimens of lung tissue, signs of desquamation of the alveolar epithelium, obstructive bronchiolitis are revealed.

The late stage is manifested by severe respiratory failure, severe symptoms of decompensated chronic pulmonary heart, diffuse ash-gray cyanosis and acrocyanosis, hypertrophic osteoarthropathy. The blood shows a high level of IgG, circulating immune complexes, characterized by a high activity of lipid peroxidation and a decrease in the activity of the antioxidant system. In the biopsy specimens of pulmonary tissue, marked sclerotic changes and structural rearrangement by the type of "cellular lung", atypical dysplasia and adenomatosis of the alveolar and bronchial epithelium are revealed.

Isolate acute and chronic variants of idiopathic fibrosing alveolitis. The acute variant is rare and is characterized by a sharply increasing respiratory insufficiency with a lethal outcome within 2-3 months. In chronic course of idiopathic fibrosing alveolitis, aggressive, persistent, slowly progressing variants are isolated. The aggressive variant is characterized by fast progressive dyspnea, exhaustion, severe respiratory failure, life expectancy from 6 months to 1 year. The persistent variant is characterized by less vivid clinical manifestations, with a life expectancy of up to 4-5 years. Slowly progressive version of idiopathic fibrosing alveolitis is characterized by a slow development of fibrosis and respiratory failure, with a life expectancy of up to 10 years.

[1], [2], [3], [4], [5]