Idiopathic hemosiderosis of the lungs

Idiopathic hemosiderosis of the lungs( Delen-Gellerstedt syndrome) is one of the rare forms of lung lesions. The disease was first described by R. Virchow in 1864. The autosomal recessive type of inheritance of the disease is assumed. In the literature there is a description of the family forms of idiopathic hemosiderosis of the lungs.

The causes and mechanisms of the development of the disease have not yet been finally established. It is assumed that there is a congenital inferiority of the capillary network of the lungs. In addition, it is believed that the basis of the disease is the formation of antibodies against lung tissue in response to the effect of an allergen. An allergic reaction that occurs in the lungs causes damage to the pulmonary capillaries.

Idiopathic pulmonary hemosiderosis is a disease predominantly of childhood. The onset of the disease most often refers to preschool age, but it can also be in children of the first years of life. The course of the disease is usually wavy: periods of exacerbation are replaced by improvements of different duration. The main clinical signs of the disease are hemoptysis, anemia, changes in the lungs. The first exacerbations of the disease often occur according to the type of acute respiratory pathology or pneumonia. Until the appearance of classic symptoms of the disease is often diagnosed with chronic pneumonia.

In the period of exacerbation, the body temperature rises, the skin is pale, sometimes acquiring a yellowish tinge. There are shortness of breath, chest pain, wet cough. In the lungs wet rales are heard.

Hemoptysis has a different intensity and duration. There can be separate blood veins in the sputum, sometimes sputum is intensely stained with blood, pulmonary bleeding is possible. The liver and spleen were exacerbated during the exacerbation period.

Radiographic changes in the lungs depend on the period of the disease, the duration of its course.

As the disease progresses, exacerbations become harder, improvements are shorter. Sometimes the process immediately acquires a heavy, rapidly progressing current.

Death in this disease comes from pulmonary heart failure or pulmonary hemorrhage.