Idiopathic pulmonary fibrosis - a diffusive intersticial progressing pulmonary fibrosis; differs exclusively pulmonary localization of process, small effect of therapy, a frequent lethal outcome. Disease is considered rare, but its frequency increases. The etiology is obscure. The autoimmune pathogenesis Is supposed. Lungs are dense, red and brown color with a network of grayish strias and the centers of a violent emphysema.

Signs, current. Leaders and, constant signs - sharply expressed, progressing and refractory to therapy a dyspnea and a cyanosis. The anoxemia accrues, the pulmonary hypertensia and a failure of a right ventricle develops. Tussis, drum-type fingers, bilateral bitter crepitant rhonchuses, a dry pleuritis are marked; the spontaneous pheumothorax is possible. Disturbances of the ventilation, progressing depression of ability of lungs to diffusion are characteristic. Radiological attributes - a diffusive intersticial fibrosis, an emphysema of lungs. Changes of a leukogram, a fervescence are caused by usually secondary infection. Current acute, subacute and chronic.