Hemosiderosis of the lungs is a very serious disease. It occurs when a massive hit of red blood cells in the tissue of the human lung. At the same time, the development of the hemosiderin pigment containing iron continues for a long time.
What is hemosiderosis?
To diagnose this disease is possible 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 are a strong cough, including blood spitting, shortness of breath, bleeding in the lungs. Fever and tachycardia may occur.
Based on an X-ray of the lungs, the doctor can make a final diagnosis. Other studies are also being conducted to confirm that the patient is suffering from this particular disease. This is a study of sputum, a biochemical blood test, in complex cases - a lung biopsy.
Treatment of the disease is carried out using corticosteroids and drugs that eliminate specific symptoms.
Manifestation of the disease
Idiopathic hemosiderosis of the lungs is also called Cehlen-Gellerstedt syndrome. This is a pathological problem that manifests itself as a result of 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. He is responsible for storing iron in the body. During various diseases, it accumulates in the tissues, which leads to problems in the patient’s health. It can also accumulate in human organs.
At its core, hemosiderin is a form that stores spare iron in the body. Because of this, the fabrics have a pronounced rusty shade. When a person develops idiopathic hemosiderosis of the lungs, up to five grams of iron can accumulate in the tissues of this organ.
The accumulation of iron in itself does not necessarily affect the functioning of the main elements of the organ, in this case the lung. However, if hemosiderosis of the lungs is accompanied by sclerosis, then functional disorders in the body are inevitable.
At risk for patients susceptible to this disease are young children and adolescents. Most often female.
Causes of the disease
Hemosiderosis of the lungs can occur in humans for various reasons. The most common is a congenital defect in the walls of the vessels of the pulmonary circulation. Because of this, in the body there is a depletion of capillaries and a temporary cessation of blood flow. As a result, red blood cells sweat, and frequent pulmonary microbleeding occurs in the lung tissue.
As a result of this, iron is released and enters the hemosiderin composition. It, in turn, is absorbed by alveolar macrophages and is deposited in large excess in the epithelium and endothelial cells. Another fatal defect also plays a fatal role. Defective connection of two organs, in this case - the bronchial arteries and veins of the lung.
Immunoallergic nature of the disease
The causes and morphogenesis of hemosiderosis of the lung can also be immunoallergic. In this case, the immune complexes cause the main damage to the walls of the pulmonary capillaries. There is a violation of their normal functioning, through the damaged vessels in the lungs in excess iron gets in, as a result, the work of the organ is seriously impaired.
Also, the excessive process of destruction of blood red blood cells, accompanied by the release of hemoglobin, which occurs in the spleen, contributes to the excessive deposition of hemosiderin in the lung tissues. As well as a high level of absorption of iron in the intestine, prolonged use of drugs with a high level of iron content.
At the initial stage, hemosiderosis of the lungs in children can occur as an idiopathic disease that is not associated with other problems. And it may be accompanied by Heiner syndrome (in the event that the body is too sensitive to proteins contained in natural cow's milk).
If hemosiderosis occurs repeatedly, then pulmonary microbleeding can lead to high blood pressure, which can become a chronic problem.
Also, this disease may be accompanied by Goodpasture syndrome. This is a lesion of the alveoli of the lungs and kidneys, accompanied by scarring. This disease is usually affected by men aged 20 to 30 years. There is an opinion about its hereditary nature.
Congestive hemosiderosis
Idiopathic hemosiderosis of the lungs in children is often provoked by infectious diseases. This can be either a mild ARVI or a more serious illness - measles, whooping cough or malaria. It can also be the effects of intoxication.
Congestive hemosiderosis develops with chronic heart problems. It can be all kinds of cardiological pathologies - heart defects, cardiosclerosis and other diseases.
Frequent causes of repeated as well as congestive hemosiderosis are prolonged exposure to low temperatures, strong physical or mental strain, or the use of certain medications.
If hemosiderosis occurs inside the lung alveoli, then traces of microbleeds are clearly visible in the pictures, and the sites of excessive deposition of hemosiderin are similar to nodules that are located from the center of the lung to its periphery.
Symptoms
Hemosiderosis can occur in several forms. Acute, subacute and relapsing. 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 given this diagnosis in the first weeks of life.
The disease manifests itself as pulmonary hemorrhage and bleeding, a feeling of respiratory failure.
During the occurrence of complications, a person develops a severe cough with sputum of a rusty color, in severe cases there may be hemoptysis. In young children, blood may be in the vomit.
During the examination, doctors pay attention to wheezing, tachycardia, fever, often the patient complains of pain in the chest and abdominal cavity, as well as in the joints. A pathological enlargement of the liver and a sharp drop in weight can also occur.
If hemoptysis lasts a long time, then the patient develops anemia, weakness and dizziness. The skin becomes pale, and characteristic yellowness appears around the eyes. At the same time, a person becomes tired and constantly hears tinnitus. A severe period of the disease can occur from several hours to 10-15 days. Therefore, there is no way to do without medical assistance.
During a period of deterioration, cough and shortness of breath may decrease, but this should not be very encouraging, you should consult a doctor immediately. During remission, complaints may be absent altogether, and a person can lead a full-fledged, healthy life.
With each exacerbation of this disease, the time of remission is reduced, but the severity of crises increases proportionally for a long time. One of the consequences of anemia is a general depletion of the body. In rare cases, even the death of the patient is possible - such a sad outcome can occur due to acute pulmonary bleeding and severe respiratory failure.
Diagnosis of hemosiderosis
In order to accurately diagnose hemosiderosis of the lungs, an expert opinion of several experts is necessary. First of all, this is a pulmonologist, a hematologist. And also the study of the clinical manifestations of the disease will be required. You can not do without the study of x-rays, analysis of sputum, blood (both general analysis and biochemical), as well as lung biopsy.
Diagnosing this disease is extremely difficult. Often months and even years pass before specialists accurately determine the diagnosis. Sometimes they put him only posthumously. The thing is that the first signs are not very specific, similar to many other diseases, and many simply do not attach importance to them. Usually these are just respiratory illnesses accompanied by coughing and anemia.
Acute stages
If doctors diagnose idiopathic hemosiderosis of the lungs, treatment is necessary. The body itself can not cope with this problem. In acute forms, the disease is accompanied by wet wheezing and heart palpitations. At the same time, the level of red blood cells is lowered in the blood, bilirubin is increased, and 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 bone marrow hyperplasia.
X-ray studies
When studying x-rays in the early stages of the disease, a decrease in the transparency of the pulmonary fields is characteristic. At the same time, at the third and fourth stages, intense darkening and foci of connective tissue with characteristic scars are observed.
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 the final diagnosis.
Hemosiderosis treatment
Successful treatment of this disease is possible only with prolonged use of a course of corticosteroids. For example, Prednisone helps treat hemosiderosis of the lungs. The drug minimizes vascular permeability and inhibits the development of autoimmune reactions.
If conservative methods of treatment are considered ineffective, then surgical methods are used. The spleen can be completely or partially removed, this 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 the disease
It is noteworthy that in the acute period and with prevention, similar drugs are prescribed. With a diagnosis of pulmonary hemosiderosis, treatment is effectively carried out using a combination of cytostatics and plasmapheresis. This reduces the appearance of new autoantibodies and helps the body cope with old ones.
Also, an effective method is the elimination of iron with urine, for this, Desferal infusions are used. In the treatment of symptoms, bronchodilators, anticoagulant drugs are used.
Doctors manage to achieve long-term remission if their patients follow a strict diet, from which all products based on cow's milk are excluded. If the disease has spilled over into the chronic stage, nitrates may be prescribed. This is especially due to chronic heart problems.
Types of hemosiderosis
Doctors distinguish several varieties of this disease. In particular, hemosiderosis of the lungs is characterized (macrodrug “Brown lung induction”). In this case, the lungs increase in size, have an extremely dense texture. As well as dark red, close to brown, whitish layers and brownish inclusions are visible in the section.
Hemosiderosis of the lung may also occur (micropreparation No. 111). In this case, brown pigments are noticeable inside the cells and beyond, which, during the laboratory Perls reaction, turn blue or greenish. They arise between the bronchi and in the cavities of the alveoli.
At the same time, the vessels of the lung of the person themselves are extremely dilated and full-blooded. The disease is accompanied by profuse hemorrhage in the septum between the alveoli. At the same time, layers of connective tissue are found in them.