Fibrosis of the lungs

Among the lung diseases, idiopathic diffuse pulmonary fibrosis takes a special place. This disease also has another name: the Hammam-Rich syndrome, after the names of the authors who described it.

Idiopathic diffuse pulmonary fibrosis refers to diseases with an autosomal dominant type of inheritance. This pathology refers to diseases that develop as a result of a violation of the immune system. At the heart of the disease is the damage to the small vessels of the lungs. At the final stage, the structure of the lungs is lost, the lung tissue is replaced with a fibrous( connective) tissue, cysts are formed. The most characteristic signs characteristic only of this disease, it is extremely difficult to distinguish. It is believed that most often the disease affects people aged 50-60 years. However, it occurs in different age groups.

The main clinical manifestation of idiopathic diffuse pulmonary fibrosis is dyspnea. Initially, it appears after physical exertion, then it is saved and at rest. The intensity of dyspnea progressively increases. As the disease progresses, cyanotic coloration of the skin - cyanosis, usually it is not clearly expressed. Cough, as a rule, dry, unproductive. Sometimes hemoptysis is observed. A characteristic of idiopathic diffuse pulmonary fibrosis is the incompatibility of dyspnea with relatively small changes in the lungs. A typical sign of the disease is a progressive loss of body weight. One of the earliest manifestations of the disease is the thickening of the nail phalanges of the fingers."Drum sticks" are sometimes found in these patients before the onset of pulmonary changes.

Special methods of investigation usually reveal a sharp decrease in pulmonary volumes( volume of inhaled and exhaled air, etc.).Radiographic examination of chest organs reveals focal shadows that correspond to the formed cysts of the lungs.

Often the process involves the lining of the lung( pleura), changes are detected in the area of ​​the roots of the lungs. With bronchography, bronchial narrowing and deformity are detected. However, this study is unsafe for this disease, and it should be avoided.

The prognosis of the disease is unfavorable. Death comes from pulmonary and cardiac failure. The average life expectancy in the chronic course of the process and the absence of treatment usually does not exceed 4-6 years. In children, especially the early age, the disease usually proceeds in acute form and within a few months can lead to the death of the child.

The main method of treatment is hormone therapy in combination with immunosuppressants. Medicines such as azathioprine and coulenyl are used. The effectiveness of treatment depends on the severity of clinical manifestations at the time of the initiation of hormone therapy.