Idiopathic fibrosing alveolitis - Symptoms

Idiopathic fibrosing alveolitis develops most often between the ages of 40 and 70, and in men it is 1.7-1.9 times more common than in women.

The most typical is a gradual, barely noticeable onset, however, in 20% of patients the disease begins acutely with an increase in body temperature and severe shortness of breath, but subsequently the body temperature normalizes or becomes subfebrile.

For idiopathic fibrosing alveolitis, the complaints of patients are extremely characteristic, a thorough analysis of which allows us to suspect this disease:

• dyspnea is the main and constant manifestation of the disease. At first, dyspnea is less pronounced, but as the disease progresses, it increases and becomes so pronounced that the patient cannot walk, take care of himself or even talk. The more severe and prolonged the disease, the more pronounced the dyspnea. Patients note the constant nature of dyspnea, the absence of attacks of suffocation, but often emphasize the inability to take a deep breath. Due to progressive dyspnea, patients gradually reduce their activity and prefer a passive lifestyle;
• cough is the second characteristic symptom of the disease, about 90% of patients complain of cough, but this is not the first symptom, it appears later, as a rule, during the period of pronounced clinical picture of idiopathic fibrosing alveolitis. Most often, the cough is dry, but in 10% of patients it is accompanied by the separation of mucous sputum;
• chest pain - observed in half of patients, it is most often localized in the epigastric region on both sides and, as a rule, intensifies with deep inhalation;
• weight loss is a characteristic symptom of idiopathic fibrosing alveolitis and usually bothers patients in the progression phase of the disease; the degree of weight loss depends on the severity and duration of the pathological process in the lungs, weight loss of 10-12 kg is possible within 4-5 months;
• general weakness, rapid fatigue, decreased performance - complaints that are typical for all patients, especially pronounced during the progressive course of the disease;
• joint pain and morning stiffness are uncommon complaints, but can be noticeable in severe cases of the disease;
• An increase in body temperature is not a typical complaint for idiopathic fibrosing alveolitis, however, according to M. M. Ilkovich and L. N. Novikova (1998), 1/3 of patients have subfebrile or febrile body temperature, most often between 10 and 13 hours. Fever indicates an active pathological process in the lungs.

An objective examination reveals the following characteristic manifestations of idiopathic fibrosing alveolitis:

• dyspnea and cyanosis of the skin and visible mucous membranes - initially observed mainly during physical exertion, and as the disease progresses, they significantly increase and become constant; these symptoms are early signs of the acute form of idiopathic fibrosing alveolitis; with severe respiratory failure, diffuse cyanosis of a gray-ash color appears; a distinctive feature of breathing is the shortening of the inhalation and exhalation phases;
• changes in the nail phalanges (thickening of the 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 in women. This symptom is more natural with pronounced activity and long duration of the disease;
• changes in sound during percussion of the lungs - dullness is characteristic over the affected area, mainly in the lower parts of the lungs;
• Characteristic auscultatory phenomena are weakening of vesicular breathing and crepitation. Weakening of vesicular breathing is accompanied by shortening of the inhalation and exhalation phases. Crepitation is the most important symptom of idiopathic fibrosing alveolitis. It is heard on both sides, mainly along the posterior and mid-axillary lines in the interscapular region, in some patients (with the most severe course of the disease) - over the entire surface of the lungs. Crepitation resembles the "cracking of cellophane". Compared with crepitation in other lung diseases (pneumonia, congestion), crepitation in idiopathic fibrosing alveolitis is more "gentle", higher in frequency, less loud, and is best heard at the end of inhalation. As the pathological process in the lungs progresses, the "gentle" timbre of crepitation can change to a more sonorous and rough one.

In advanced idiopathic fibrosing alveolitis, another characteristic sign appears - "squeaking", which resembles the sound of friction or turning of a cork. The phenomenon of "squeaking" is heard on inspiration and mainly over the upper pulmonary fields and, mainly, in pronounced pleuro-pneumosclerotic processes.

In 5% of patients, dry wheezing may be heard (usually with the development of concomitant bronchitis).

The course of idiopathic fibrosing alveolitis

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

The main complications of idiopathic fibrosing alveolitis are chronic pulmonary heart disease, severe respiratory failure with the development of hypoxemic coma at the end of the disease. Less common are pneumothorax (with a formed "honeycomb lung"), pulmonary embolism, and exudative pleurisy.

From the moment of diagnosis of idiopathic fibrosing alveolitis, patients live about 3-5 years. The main causes of death are severe cardiac and respiratory failure, pulmonary embolism, secondary pneumonia, and lung cancer. The risk of developing lung cancer in patients with IFA is 14 times higher than in the general population of the same age, gender, and smoking history. Along with this, it is reported that recovery is possible in such forms of idiopathic fibrosing alveolitis as desquamative interstitial, acute interstitial, and nonspecific interstitial pneumonia when using modern methods of therapy.

A. E. Kogan, B. M. Kornev, Yu. A. Salov (1995) distinguish early and late stages of idiopathic fibrosing alveolitis.

The early stage is characterized by grade 1 respiratory failure, mild immune-inflammatory reaction, diffuse increase in interstitial pattern without significant deformation. Patients complain of sweating, arthralgia, weakness. There is no cyanosis yet. "Tender" crepitation is heard in the lungs, there is no hypertrophic osteoarthropathy (symptom of "drumsticks" and "watch glasses"). In lung tissue biopsies, exudative and exudative-proliferative processes in the interstitium predominate, signs of desquamation of the alveolar epithelium and obstructive bronchiolitis are revealed.

The late stage is manifested by severe respiratory failure, pronounced symptoms of decompensated chronic pulmonary heart disease, diffuse ash-gray cyanosis and acrocyanosis, hypertrophic osteoarthropathy. High levels of IgG and circulating immune complexes are detected in the blood, high activity of lipid peroxidation and decreased activity of the antioxidant system are characteristic. Biopsies of lung tissue reveal pronounced sclerotic changes and structural reorganization of the "honeycomb lung" type, atypical dysplasia and adenomatosis of the alveolar and bronchial epithelium.

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

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