Idiopathic pulmonary hemosiderosis is a lung lesion caused by the massive influx of red blood cells into the lung tissue with the deposition of the iron–containing pigment hemosiderin in it. The course of the disease is accompanied by crises, during which cough, hemoptysis, shortness of breath, fever, tachycardia, respiratory failure, pulmonary bleeding and anemia occur. The diagnosis of hemosiderosis is confirmed by the data of lung radiography, sputum microscopy, blood test, spirography, lung biopsy. With idiopathic pulmonary hemosiderosis, corticosteroids and symptomatic drugs are used, splenectomy is performed if necessary.
Idiopathic pulmonary hemosiderosis (Celen-Gellerstedt syndrome) is a pathology characterized by increased deposition of hemosiderin in the alveoli and stroma of the lungs, resulting from recurrent pulmonary hemorrhages and extensive hemolysis of erythrocytes. Hemosiderin is one of the forms of spare iron in the body, which accumulates in the foci of hemorrhages and gives the tissues a “rusty” hue. The morphological picture of pulmonary hemosiderosis in pulmonology is described as “brown lung induration”. Up to 5 g of iron can be deposited in the lung tissue during hemosiderosis. The accumulation of hemosiderin in the lungs does not damage the parenchyma, but when combined with sclerosis leads to functional disorders. Idiopathic pulmonary hemosiderosis is observed mainly in children and young patients, more often female.
Primary idiopathic pulmonary hemosiderosis may occur as an independent type (idiopathic) or be associated with Gainer syndrome caused by hypersensitivity to cow’s milk proteins. In secondary Idiopathic pulmonary hemosiderosis, erythrocyte diapedesis and diffuse alveolar bleeding develop due to chronic pulmonary hypertension (in particular, mitral stenosis) or against the background of thrombocytopenic purpura and vasculitis. A combination of idiopathic pulmonary hemosiderosis with glomerulonephritis (Goodpascher syndrome) is possible. The deposition of hemosiderin in the lungs is facilitated by accelerated hemolysis of erythrocytes in the spleen, metabolic disorders of iron-containing pigments, excessive absorption of iron in the intestine, frequent hemotransfusion, prolonged intake of large doses of iron-containing drugs.
In the etiology of idiopathic pulmonary hemosiderosis, the role of the infectious factor (transferred ARVI, measles, whooping cough, malaria, etc.) and intoxication has been proven. The presence of chronic venous stasis against the background of cardiac pathology (decompensated heart defects, cardiosclerosis) has a tendency to develop congestive idiopathic pulmonary hemosiderosis. Triggers for the manifestation of idiopathic pulmonary hemosiderosis can be exposure to low temperatures, excessive mental and physical stress, the use of certain medications (NSAIDs, paracetamol, diuretics).
In the selective deposition of hemosiderin in lung tissue, a certain role is played by a congenital defect in the elastic fibers of the walls of the vessels of the small circulatory circle. As a result, thinning and dilation of capillaries and pronounced blood stasis occur, leading to sweating of red blood cells, frequent pulmonary microbleeds. Iron released during hemolysis does not participate in erythropoiesis, but is included in the composition of hemosiderin, which is absorbed by alveolar macrophages, is deposited in excess in epithelial and endothelial cells, filling the lumen of the alveoli, bronchi and stroma of the lungs. Considerable importance is given to the congenital inferiority of anastomoses between bronchial arteries and pulmonary veins.
The immunoallergic nature of idiopathic idiopathic pulmonary hemosiderosis has been confirmed, in which, under the influence of a certain sensitizing factor, autoantibodies to the components of the alveoli and vessels of the microcirculatory bed are produced. The resulting immune complexes affect, first of all, the walls of the pulmonary capillaries, causing their destruction and facilitating extravasation through damaged vessels into the pulmonary parenchyma.
With idiopathic pulmonary hemosiderosis, traces of hemorrhages with hemolysis of erythrocytes and an abundance of macrophages filled with lumps of hemosiderin are visible inside the alveoli and in the pulmonary interstitium. Hyperplasia of the alveolar epithelium, thickening of the interalveolar septa is detected. The sites of hemosiderin deposition have the appearance of small macroscopically visible nodules scattered from the center of the lungs to the periphery. The impregnation of the vessel walls with iron salts causes their thinning, fragmentation and degenerative transformation of the elastic tissue of the lung.
Celen-Gellerstedt syndrome can occur in the form of an acute, subacute or recurrent process with a long-term wave-like course and a change of periods of exacerbations (crises), subcompensation and remission. In children, it usually manifests itself at the age of 3-7 years, although the first signs are possible already in the first months of a child’s life. The clinic of idiopathic pulmonary hemosiderosis is characterized by pulmonary hemorrhages and bleeding with the development of hypochromic anemia and respiratory failure.
The crisis is accompanied by a moist copious cough with “rusty” sputum, sometimes with blood, the volume of loss of which can reach the degree of severe bleeding; vomiting with an admixture of blood is possible in young children. There is shortness of breath, wheezing, tachycardia, fever (often febrile), pain behind the sternum and in the abdominal cavity, joints, the formation of a pneumonic status, splenic and hepatomegaly, weight loss are not uncommon.
Prolonged hemoptysis leads to the development of severe anemia with severe weakness, dizziness, pallor and cyanosis of the skin, jaundice of the sclera, fatigue, tinnitus. The acute period can last from several hours to 1-2 weeks. At the stage of subcompensation, cough and shortness of breath gradually decrease; during remission, the condition of patients is relatively well, there are practically no complaints, working capacity is maintained. Subacute forms of idiopathic pulmonary hemosiderosis do not have bright exacerbations. Gainer’s syndrome occurs against the background of hypertrophy of nasopharyngeal tissues with manifestations of rhinitis and recurrent otitis media, various disorders of the gastrointestinal tract.
With each recurring exacerbation of idiopathic pulmonary hemosiderosis, the duration of remissions shortens and the severity of crises increases. Prolonged anemia causes general exhaustion of the body. The progression of idiopathic pulmonary hemosiderosis contributes to the development of recurrent pneumothorax, diffuse pneumofibrosis, pulmonary hypertension and pulmonary heart. The addition of infarct-pneumonia aggravates the course of hemosiderosis. A fatal outcome is possible as a result of acute pulmonary bleeding and an increase in respiratory failure.
Diagnosis of idiopathic pulmonary hemosiderosis is carried out with the participation of a pulmonologist, hematologist, geneticist, taking into account anamnesis, clinical manifestations, X-ray data, sputum microscopy, general and biochemical blood analysis, spirography, lung biopsy. Disease is often diagnosed months and years after the onset, and not even always in vivo. The first signs are not specific, frequent respiratory diseases with cough, anemia are noted in the medical history.
In the acute stage of a patient with idiopathic pulmonary hemosiderosis, zones of shortening of the pulmonary sound, the presence of inhomogeneous, diffuse wet wheezing, weakening and increased heart tones are determined. Hypochromic anemia is recorded in the blood (a decrease in the level of Hb, erythrocytes), reticulocytosis, an increase in indirect bilirubin and gamma-globulin, a decrease in serum iron levels; during a crisis – leukocytosis, an increase in ESR; in the later stages – periodic compensatory polycythemia. The detection of hemosiderophages and erythrocytes in sputum flushes or gastric lavage waters (in children) serves as an important criterion for the diagnosis of idiopathic pulmonary hemosiderosis. The myelogram shows a decrease in sideroblasts and an increase in erythropoiesis.
On the lung x-ray at stage I – II of the disease, a decrease in the transparency of the pulmonary fields, bilateral symmetrical multiple miliary foci are visible, at stage III – IV – larger, sometimes merging intense darkening and foci of interstitial fibrosis. The spontaneous disappearance of old and the appearance of new focal shadows is characteristic. Spirography determines respiratory insufficiency of restrictive type, reduced diffusion of gases; ECG – signs of myocardiodystrophy. An open or puncture lung biopsy helps to confirm the diagnosis, revealing an increase in the level of iron (100-200 times) and hemosiderophages in lung tissue preparations, interstitial fibrosis.
Idiopathic pulmonary hemosiderosis is important to differentiate with occupational siderosis, hemochromatosis, miliary pulmonary tuberculosis, carcinomatosis, bronchial cancer, sarcoidosis, silicosis, Goodpasture syndrome, Rendu-Osler disease, pneumonia, hemolytic anemia of other genesis.
With idiopathic pulmonary hemosiderosis , positive dynamics and periodic remissions are achieved by prescribing a long course of corticosteroids (prednisone). This reduces vascular permeability and inhibits the development of autoimmune reactions.
In the acute period and as a preventive measure, the use of a combination of cytostatics (azathioprine, cyclophosphane) and plasmapheresis is effective, helping to inhibit the neoplasm of autoantibodies and remove previously developed ones. Desferal infusions are indicated to eliminate excess iron in the urine. Anticoagulant and antiplatelet agents (heparin, dipyridamole, pentoxifylline), iron preparations, bronchodilators are used as symptomatic treatment. Therapy conducted against the background of a strict dairy-free diet helps to achieve long-term remission in patients with precipitins to cow’s milk. In the case of a chronic pulmonary heart, nitrates are prescribed to relieve pulmonary hypertension. If conservative therapy is ineffective, splenectomy is performed, which allows to significantly prolong remissions, reduce the severity of crises and increase the patient’s life expectancy by 5-10 years or more.
Prognosis and prevention
Idiopathic pulmonary hemosiderosis refers to diseases with a rather unfavorable prognosis. The progression of idiopathic pulmonary hemosiderosis threatens the development of serious complications – pulmonary hypertension, extensive pulmonary bleeding, severe respiratory failure. Each crisis carries a risk of death. The average life expectancy of patients with idiopathic pulmonary hemosiderosis is 3-5 years. The prevention of the disease can be timely treatment of concomitant vascular, cardiac, hematological, allergic pathology, control during hemotransfusion, taking iron preparations and other medicines.
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