Hamman-rich syndrome

Hamman-rich syndromeHAMMAN - RICH SYNDROME - progressive diffuse interstitial pulmonary fibrosis; it is exclusively pulmonary localization process, the small effect of therapy, frequent fatal outcome. The disease is considered rare, but the frequency increases. The etiology is unclear. It is assumed autoimmune pathogenesis. The main pathomorphological substrateless-capillary unit, resulting in impaired diffusion capacity of the lungs. Typical serous-fibrinous impregnation of the alveolar walls, accumulation in the alveoli-rich fibrin and exudate macrophages expressed the growth of connective tissue and its hardening, hyaline membranes in the alveoli; light dense, red-brown with a network of grayish stripes and pockets of bullous emphysema. The symptoms for. Leading and persistent symptoms are pronounced, progressive and resistant to treatment, dyspnea and cyanosis. Increasing hypoxemia, pulmonary hypertension and right ventricular failure. There are cough, drum fingers, bilateral bitter traiterous wheezing, dry pleurisy; possible spontaneous pneumothorax. Typical restrictive type of violation ventilation, progressive decline in lung diffusion capacity. X-ray evidence of diffuse interstitial fibrosis, pulmonary emphysema. Changes leukogram, proteinogram, erythrocyte sedimentation rate, increased body temperature usually due to secondary infection. For acute, subacute and chronic. Treatment. In the early stages of the disease designate prednisolone 40 mg/day with gradual dose reduction while achieving clinical effect. In the late period of the disease and rapidly progressive course - corticosteroids, combining them with immunosuppressants (azathioprine 2.5 mg/kg / day for 2 months, then 1.5 mg/kg per day for 1 year or more) and penitsillaminom, or kuprenilom (300 mg/day, then gradually increase the dose to 1.8 g/day, followed by reduction to support 300 mg/day). Concurrently used drugs, potassium verospiron, pyridoxine..



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