Hammam-Rich syndrome

Hammena-Rich syndrome - diffuse interstitial progressive pulmonary fibrosis; Differs exclusively pulmonary localization of the process, a small effect of therapy, a frequent lethal 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. Serous-fibrinous impregnation of alveolar septa, accumulation in the alveoli of fibrin-rich and macrophage exudates, marked expansion of connective tissue and its sclerosis, hyaline membranes in the alveoli; Lungs are dense, red-brown color with a network of grayish stripes and foci of bullous emphysema.

Symptoms, course. Leading and persistent symptoms are pronounced, progressive and resistant to therapy shortness of breath and cyanosis. Increases in hypoxemia, pulmonary hypertension and right ventricular failure develop. Cough, "tympanic fingers", bilateral crackling crepitations, dry pleurisy are noted; Spontaneous pneumothorax is possible. A restrictive type of ventilation disturbance is characteristic, a progressive decrease in the diffusive capacity of the lungs. X-ray signs-diffuse interstitial fibrosis, emphysema of the lungs. Changes in the leukogram, proteinogram, ESR, body temperature increase are usually caused by a secondary infection. The course is acute, subacute and chronic.

Treatment. In the early stages of the disease, prednisolone is prescribed up to 40 mg / day with a gradual decrease in dose when the clinical effect is achieved. In the late period of the disease and with rapidly progressing flow, corticosteroids, combining them with immunosuppressants (azathioprine 2.5 mg / kg per day for 2 months, then 1.5 mg / kg per day for 1 year and more) and penicillamine, Or kurenyl (300 mg / day, then gradually increase the dose to 1.8 g / day, followed by a decrease to a maintenance dose of 300 mg / day). At the same time, potassium, verospilone, pyridoxine are used.