pulmonary fibrosis

August 12, 2017 17:51 | Genetic Diseases

Among lung disease occupies a special place diffuse idiopathic pulmonary fibrosis.The disease also has another name: Hamman Rich syndrome, by author's name, describe it.

diffuse idiopathic pulmonary fibrosis refers to diseases with autosomal dominatnym type of inheritance.Considered pathology refers to diseases, developing as a result of dysfunction of the immune system.At the heart disease is damage to the small vessels of the lungs.At the final stage of lung structure is lost, lung tissue is replaced by fibrous (connective) tissue, cysts are formed.The most characteristic features peculiar only to this disease, highlight the extremely difficult.It is believed that the disease often affects people between the ages of 50-60 years.However, it is found in various age groups.

main clinical manifestation of diffuse idiopathic pulmonary fibrosis is shortness of breath.At first it appears after exercise, and then stored at ease.The intensity of the dyspnea progressively increases.As the disease appears blu

ish color of the skin - cyanosis, usually expressed mild.Cough, usually dry, nonproductive.Sometimes there is hemoptysis.Characteristic of diffuse idiopathic pulmonary fibrosis is considered a mismatch dyspnea relatively small changes in the lungs.A typical sign of the disease - a progressive loss of body weight.One of the earliest manifestations of the disease - a thickening of nail phalanges."Drumsticks" are sometimes found in these patients before the advent of pulmonary changes.

During the special methods of investigation usually reveals a sharp decline in lung volume (the volume of air inhaled and exhaled, and others.).When X-ray examination of the chest revealed patchy shadows that correspond to the generated light cysts.

Often the process involves the shell of the lung (pleura), to detect changes in the roots of the lungs.When bronhografii found bronchoconstriction, their deformation.However, this study is not safe in this disease, and should be avoided.

forecast unfavorable disease.Death occurs from pulmonary and cardiac failure.The life expectancy of the chronic course of the process and the absence of treatment usually does not exceed 4-6 years.In children, especially young children, the disease usually occurs in acute form and can lead to death of the child during the several months.

main method of treatment is hormone therapy in combination with immunosuppressants.They are used such drugs as azathioprine and kuprenil.The effectiveness of treatment depends on the severity of clinical manifestations at the beginning of therapy, hormonal therapy.