Hummana - rich syndrome

Hummana - rich syndrome (L. Hamman, A. Rich) - progressive diffuse interstitial fibrosis of the lungs.
The etiology is not quite clear. Most researchers relates syndrome Hummana - rich to the group of allergic diseases, noting that it can develop in people with a heightened sensitivity when exposed to various stimuli (chemical, pharmaceutical and others). Some authors refer syndrome Hummana - rich to the kollagenozov.
Pathomorphological changes occur first kind millionarin swelling to the deposition of fibrin; then in millionarmy partitions intense proliferation of connective tissue with subsequent formation of fibrosis. There metaplasia of the alveolar epithelium with the formation of hyaline membranes. Simultaneously develop emphysema and small cysts in the lung parenchyma.
The clinical picture is not sufficiently defined. Often there is a sharp discrepancy between objective data and severity of disease. Clinical recognition of the syndrome Hummana - rich is essentially a diagnosis is the final stage of the disease. The main complaints of the patients are shortness of breath (often suddenly coming), cough, dry or with a moderate amount of sputum, sometimes coughing up blood, chest pain, increasing weakness. The temperature is subfebrile or febrile. Marked cyanosis, tachycardia, shortness of breath not only when moving, but alone (respiratory rate reaches 40 or more per minute). These functional disorders associated with alveolar-capillary unit, due to the thickening millionarmy partitions, which impedes the diffusion of oxygen. In light listens scattered fine bubble moist rales. In a later stage of the disease is marked thickening of the terminal phalanges in the form of "drum sticks", hypertrophy of the right ventricle of the heart deviation of the electric axis to the right and progressive circulatory failure. Blood test usually does not present any special features, with the exception of hypergammaglobulinemia. When ray study of patients with the syndrome Hummana - rich mainly observed picture of interstitial sclerosis and emphysema with the presence of scattered different size and density of focal shadows.
The course of the disease varies: in some cases it quickly (within a few weeks or months) leads to death, in other proceeds chronically (6-8 years). Death occurs when events progressive lung and heart failure.
The diagnosis of the syndrome Hummana - rich very difficult. It should be considered if the clinical picture does not fit into the usual diffuse pneumosclerosis. To differentiate have with hematogenous disseminated pulmonary tuberculosis, pneumoconiosis, pneumonectomy, sarcoidosis, cystic easy, cancer lymphangitis, congestive easy. Great value for diagnosis have data lifetime puncture biopsy.
The radical methods of treatment there. Therapy steroid hormones in most cases gives remission, but they are seldom long. Also used bronchodilator, heart drugs, oxygen therapy.