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Idiopathic diffuse pulmonary fibrosis (Hamman-Rich syndrome, idiopathic fibrosing alveolitis - ELISA)



Pathogenesis. The pathogenesis of ELISA is considered as an autoimmune process; it is rare in childhood, more often in people 50-60 years old.

The clinical picture. Shortness of breath (mostly difficult to breathe), cough (dry, unproductive), mismatch of shortness of breath with relatively small physical changes in the lungs, nail plates in the form of “drum sticks”, sometimes hemoptysis, auscultation, a few tiny creaking wet rales (“cracking cellophane”), hypoxemia, hypercapnia.

An X-ray examination determines the diffuse enhancement of the pulmonary pattern, the presence of focal shadows; the symptom of “frosted glass” is a diffuse decrease in the transparency of the lung tissue.

With bronchography, narrowing of the bronchi, their deformation.

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Idiopathic diffuse pulmonary fibrosis (Hamman-Rich syndrome, idiopathic fibrosing alveolitis - ELISA)

  1. Idiopathic Fibrosing Alveolitis
    The term "fibrosing alveolitis" refers to a group of lung diseases that are characterized by interstitial inflammation, thickening of the walls of the alveoli and their destruction and pneumosclerosis. Pneumosclerosis is accompanied by more than 160 diseases, some of which are listed in the table. 8.2. In 50% of cases, the cause of fibrosing alveolitis remains unknown. In such cases, they speak of idiopathic
  2. Idiopathic fibrosing alveolitis.
    Idiopathic fibrosing alveolitis (ELISA) is not a single nosology. It is believed that currently ELISA represents a group of diseases that includes the following units: classical interstitial pneumonia, "nonspecific" interstitial pneumonia, desquamative pneumonia, obliterating bronchiolitis with organizing pneumonia, and giant cell interstitial pneumonia. Early
  3. Idiopathic pulmonary fibrosis
    Idiopathic pulmonary fibrosis belongs to a group of diseases whose nature is unclear. Morphological manifestations are represented by diffuse interstitial inflammation and fibrosis. Hypoxia and cyanosis occur. There are at least 20 synonyms for idiopathic pulmonary fibrosis, among which three are especially popular: chronic interstitial (interstitial) pneumonitis, Hamman-Rich syndrome
  4. Idiopathic hemosiderosis of the lungs (Cehlen-Gellerstedt syndrome)
    The basis of the disease is the formation of antipulmonary antibodies in response to exposure to a sensitizing agent. The resulting immune complexes are fixed on the basement membranes of the alveoli and pulmonary capillaries and cause damage to the lung tissue. An allergic reaction that develops on the territory of the shock organ causes damage to the pulmonary capillaries, diapedesis and destruction of red blood cells with
  5. Hemorrhagic syndrome with idiopathic thrombocytopenic purpura
    Hemorrhagic syndrome with idiopathic thrombocytopenic purpura (ITP) - spontaneous hemorrhages and bleeding from the skin and mucous membranes, which are based on a sharp decrease in platelet count. The most common triggers for bleeding in ITP are: 1. Viral and bacterial infections. 2. Preventive vaccinations. 3. Household injuries. 4. Medicines
  6. Idiopathic ventricular fibrillation
    This category includes patients who survived cardiac arrest due to ventricular fibrillation, but who did not reveal changes in the structural and functional state of the myocardium. The prospect of investigating the pathogenesis of idiopathic ventricular fibrillation is associated with the identification of genetic markers of sudden cardiac death, as well as structural disorders at the molecular level. In countries
  7. Idiopathic thrombocytopenic purpura
    Idiopathic thrombocytopenic purpura (ITP), or Werlhof’s disease (named after the author who described this disease in 1735), is a pathology associated with a decrease in the number of platelets in peripheral blood that cannot be associated with any particular exogenous etiological factor. EPIDEMIOLOGY. ITP belongs to the group of hemorrhagic diathesis and is
  8. Idiopathic myelofibrosis
    Idiopathic myelofibrosis is a chronic neoplastic clonal myeloproliferative disease, manifested by early, significant bone marrow fibrosis. It is characterized by progressive splenomegaly due to portal hypertension and myeloid metaplasia, as well as a leukoerythroblastic picture of peripheral blood: moderate neutrophilic leukocytosis, stab shift,
  9. Differential diagnosis of idiopathic dilated cardiomyopathy
    Due to the non-specificity of the clinical picture of idiopathic DCMP and the data of instrumental studies, the recognition of this disease in some cases presents a certain complexity and is associated with a large number of diagnostic errors. Therefore, with DCMP, as, perhaps, with no other nosology, differential diagnosis is of great importance, and the formulation of this diagnosis
  10. Idiopathic thrombocytopenia
    Idiopathic thrombocytopenia (IT) is one of the most common disorders of the platelet link of the blood coagulation system. It should be borne in mind that platelet deficiency leads to a violation of the trophism of the endothelial lining of blood vessels, which will lead to a violation of the vascular component of the blood coagulation system. IT develops as a result of exposure to antiplatelet antibodies. Main place of their
  11. Idiopathic cases
    A small but rather important group of disorders can be classified as idiopathic cases. However, without a complete pathological examination of the heart, such a diagnosis cannot be considered established, except for a short time. On the other hand, a pathological study can be very impractical, since a similar violation is usually observed in patients with syndromes
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