Hemosiderosis of the lungs is a very serious disease. It occurs when red blood cells enter the human lung tissue massively. At the same time, the development of the hemosiderin pigment containing iron continues for a long time.
What is hemosiderosis?
This disease can be diagnosed only on the basis of an x-ray, however, there are many symptoms by which its presence can be assumed. Distinctive signs that the patient has hemosidercosis of the lungs is a strong cough, up to coughing up blood, shortness of breath, bleeding in the lungs. Fever and tachycardia may occur.
Based on the x-ray of the lungs, the doctor can make a definitive diagnosis. Other studies are also being conducted to confirm that the patient suffers from this particular disease. This is the study of sputum, a biochemical blood test, in difficult cases - a lung biopsy.
The disease is treated with corticosteroids and medications that address specific symptoms.
Manifestation of disease
Idiopathic pulmonary hemosiderosis is also called Celen-Gellerstedt syndrome. ita pathological problem that manifests itself due to the deposition of a special pigment hemosiderin in the lungs of a person. The main feature of this pigment is that it consists of iron oxide. It is responsible for storing iron in the body. During various diseases, it accumulates in the tissues, which leads to he alth problems for the patient. It can also accumulate in human organs.
At its core, hemosiderin is a form that stores reserve iron in the body. Because of this, the fabrics have a pronounced rusty tint. When a person develops idiopathic pulmonary hemosiderosis, up to five grams of iron can accumulate in the tissues of this organ.
In itself, the accumulation of iron will not necessarily affect the functioning of the main elements of the organ, in this case the lung. However, if pulmonary hemosiderosis is accompanied by sclerosis, then functional disorders in the body are inevitable.
In the risk group of patients susceptible to this disease are young children and adolescents. Most often female.
Causes of disease
Hemosiderosis of the lungs can manifest itself in a person for various reasons. The most common is a congenital defect in the walls of the vessels of the pulmonary circulation. Because of this, depletion of capillaries occurs in the body and a temporary cessation of blood flow. As a result, red blood cells sweat, and frequent pulmonary microhemorrhages occur in the lung tissues.
As a result of this ironreleased into hemosiderin. It, in turn, is taken up by alveolar macrophages and deposited in epithelium and endothelial cells in great excess. Another congenital defect also plays a fatal role. Inadequate connection of two organs, in this case, the bronchial arteries and veins of the lung.
Immunoallergic nature of the disease
Causes and morphogenesis of hemosiderosis of the lung can also be immunoallergic. In this case, immune complexes cause the main damage to the walls of the pulmonary capillaries. There is a violation of their normal functioning, iron enters the lungs in excess through damaged vessels, as a result, the work of the organ is seriously disrupted.
Also, excessive deposition of hemosiderin in the lung tissues contributes to the rapid process of destruction of red blood cells, accompanied by the release of hemoglobin, which occurs in the spleen. As well as high levels of iron absorption in the intestines, long-term use of drugs with a high level of iron.
At the initial stage, hemosiderosis of the lungs in children may occur as an idiopathic disease, not associated with other problems. Or it may be accompanied by Gainer's syndrome (in the event that the body is too sensitive to the proteins contained in natural cow's milk).
If hemosiderosis occurs repeatedly, pulmonary microbleeding can lead to high blood pressure, which can become a chronic problem.
Also, this disease may be accompanied byGoodpasture's syndrome. This is a lesion of the alveoli of the lungs and kidneys, accompanied by their scarring. This disease usually affects men between the ages of 20 and 30. There is an opinion about its hereditary nature.
Congestive hemosiderosis
Idiopathic pulmonary hemosiderosis in children is often provoked by infectious diseases. It can be either a mild SARS or a more serious illness - measles, whooping cough or malaria. It can also be the consequences of intoxication of the body.
Congestive hemosiderosis develops in chronic heart problems. It can be all kinds of cardiological pathologies - heart defects, cardiosclerosis and other diseases.
Repeated and congestive hemosiderosis are often caused by prolonged exposure to low temperatures, severe physical or mental stress, and the use of certain medications.
If hemosiderosis occurs inside the alveoli of the lungs, then the traces of microhemorrhages are clearly visible on the pictures, and the places of excessive deposition of hemosiderin look like nodules that are located from the center of the lung to its periphery.
Symptoms
Hemosiderosis can occur in several forms. Acute, subacute and recurrent. If this disease manifests itself in children, then most often it occurs even in preschool age, starting at the age of three. However, there are patients who were diagnosed with this disease in the first weeks of life.
The disease manifests itself with pulmonary hemorrhages andbleeding, feeling short of breath.
During the occurrence of complications, a person develops a severe cough with rusty sputum, in severe cases there may be hemoptysis. Young children may vomit blood.
During the examination, doctors pay attention to wheezing, tachycardia, fever, often the patient complains of pain in the chest and abdomen, as well as in the joints. There may also be a pathological enlargement of the liver and a sharp drop in weight.
If hemoptysis continues for a long time, the patient develops anemia, weakness and dizziness. The skin becomes pale, and a characteristic yellowness appears around the eyes. At the same time, a person quickly gets tired and constantly hears tinnitus. The severe period of the disease can last from several hours to 10-15 days. Therefore, medical care is indispensable here.
During the worsening of the condition, cough and shortness of breath may decrease, but this should not be very encouraging, you need to see a doctor immediately. During remission, there may be no complaints at all, and a person can lead a full-fledged working life.
With each exacerbation of this disease, the time of remissions is reduced, but the severity of crises proportionally increases for a long time. One of the consequences of anemia is the general exhaustion of the body. In rare cases, even the death of the patient is possible - such a sad outcome can happen due to acute pulmonary bleeding and severe respiratory failure.
Diagnosis of hemosiderosis
In order to accurately diagnose hemosiderosis of the lungs, you need an expert opinion from several specialists. First of all, this is a pulmonologist, a hematologist. It will also require a study of the clinical manifestations of the disease. You can not do without the study of x-rays, sputum analysis, blood (both general analysis and biochemical), as well as lung biopsy.
Diagnosing this disease is extremely difficult. It often takes months or even years for specialists to accurately determine the diagnosis. Sometimes they put it only posthumously. The thing is that the first signs are not specific, similar to many other diseases, and many simply do not attach importance to them. Usually these are just respiratory diseases accompanied by cough and anemia.
Acute stages
If doctors diagnose idiopathic pulmonary hemosiderosis, treatment is necessary. The body itself can not cope with this problem. In acute forms, the disease is accompanied by moist rales and palpitations. At the same time, the level of erythrocytes in the blood is reduced, bilirubin is increased, the level of serum iron in the blood is minimal.
During crises, leukocytosis occurs, the ESR level rises, which most often indicates the course of inflammatory processes. In the later stages of the development of the disease, polycythemia appears. This is a benign tumor process, accompanied by hyperplasia of bone marrow cells.
X-ray studies
When studying X-raypictures in the early stages of the disease is characterized by a decrease in the transparency of the lung fields. At the same time, intensive blackouts and foci of connective tissue with characteristic scars are observed at the third and fourth stages.
Often new focal shadows appear, while the old ones disappear. On spirography, respiratory failure is noticeable, on the electrocardiogram - myocardiostrophy. A lung biopsy helps to make a definitive diagnosis.
Hemosiderosis treatment
Successful treatment of this disease is possible only with a long-term course of corticosteroids. For example, "Prednisolone" helps to cure hemosiderosis of the lungs. The drug minimizes vascular permeability and inhibits the development of autoimmune reactions.
If conservative methods of treatment are recognized as ineffective, then surgical methods are used. The spleen can be completely or partially removed, which can significantly reduce the risk of remission. The likelihood of crises will also decrease, and the patient's life expectancy after such an operation will increase by 7-10 years.
Drugs in the acute period of disease development
It is noteworthy that in the acute period and for prevention, similar drugs are prescribed. With the diagnosis of "hemosiderosis of the lungs", treatment is effectively carried out using a combination of cytostatics and plasmapheresis. This reduces the production of new autoantibodies and helps the body deal with old ones.
Also an effective method is the elimination of iron in the urine, for this, Desferal infusions are used. During treatmentsymptoms, bronchodilators, anticoagulants are used.
Long-term remission can be achieved by doctors when their patients follow a strict diet, from which all products based on cow's milk are excluded. If the disease has flowed into the chronic stage, it is possible to prescribe nitrates. This is especially associated with chronic heart problems.
Types of hemosiderosis
Doctors distinguish between several varieties of this disease. In particular, hemosiderosis of the lungs is characterized (macropreparation "Brown induration of the lungs"). In this case, the lungs increase in size, have an extremely dense texture. As well as a dark red color, close to brown, whitish layers and brownish inclusions are noticeable in the section.
Hemosiderosis of the lung can also occur (micropreparation No. 111). In this case, brown pigments are visible inside and outside the cells, which become blue or greenish during the laboratory Perls reaction. They occur between the bronchi and in the cavities of the alveoli.
At the same time, the vessels of the human lung are extremely dilated and full-blooded. The disease is accompanied by profuse hemorrhages in the septum between the alveoli. At the same time, layers of connective tissue are found in them.
