Hammerich syndrome (idiopathic pulmonary fibrosis)

- diffuse interstitial progressive pulmonary fibrosis; differs exclusively in the pulmonary localization of the process, a small effect of therapy, and a frequent fatal outcome. The disease is considered rare, but its frequency increases. 

The etiology is unclear. Autoimmune pathogenesis is assumed. The main pathomorphological substrate is the alveolar-capillary block, which leads to a violation of the diffusion function of the lungs. Characterized by serous-fibrinous impregnation of the alveolar partitions, accumulation in the alveoli of exudate rich in fibrin and macrophages, pronounced growth of connective tissue and its sclerosis, hyaline membranes in the alveoli; the lungs are dense, red-brown with a network of grayish stripes and foci of bullous emphysema.

Symptoms, course. Leading and persistent symptoms-pronounced, progressive and resistant to therapy dyspnea and cyanosis. Hypoxemia increases, pulmonary hypertension and right ventricular failure develop. There is a cough, "drum fingers", bilateral crackling crepitating wheezes, dry pleurisy; spontaneous pneumothorax is possible. 

A restrictive type of ventilation violation and a progressive decrease in the diffusion capacity of the lungs are characteristic. 

Radiological signs — diffuse interstitial fibrosis, emphysema of the lungs. Changes in the leukogram, proteinogram, ESR, and body temperature are usually caused by a secondary infection. 

The course is acute, subacute and chronic.

Treatment. In the early stages of the disease, prednisoloi are prescribed up to 40 mg/day with a gradual reduction in the dose when achieving a clinical effect. In the late period of the disease and with a rapidly progressive course, glucocorticoids are combined with immunosuppressants (azathioprine 2.5 mg/kg per day for 2 months, then 1.5 mg/kg per day for 1 year or more) and penicillamine, or kuprenil (300 mg/day, then gradually increase the dose to 1.8 g/day, followed by a reduction to the maintenance — 300 mg/day). At the same time, drugs of potassium, veroshpiron, pyridoxine are used.