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Acute interstitial pneumonia (Hammain-Rich syndrome)

 
, medical expert
Last reviewed: 23.04.2024
 
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Acute interstitial pneumonia (Hammen-Rich syndrome) is an idiopathic variant of acute respiratory distress syndrome.

Acute interstitial pneumonia affects practically healthy men and women usually over the age of 40 with the same frequency.

trusted-source[1], [2], [3]

What causes acute interstitial pneumonia (Hammain-Rich syndrome)?

Acute interstitial pneumonia is differentiated histologically according to the presence of organized diffuse damage to the alveoli and nonspecific changes that occur also in other variants of lung lesions not associated with idiopathic interstitial pneumonia. A sign of organized diffuse alveolar damage is a widespread pronounced edema of alveolar septa with infiltration of their inflammatory cells; proliferation of fibroblasts; the presence of single hyaline membranes and their thickening. The septum is lined with atypical, hyperplastic pneumocytes of type II, and the air spaces are collapsed. Thrombi are formed in small arteries, which are not specific.

Symptoms of acute interstitial pneumonia (Hammain-Rich syndrome)

Acute interstitial pneumonia (Hammen-Rich syndrome) is manifested by the sudden development of fever, cough and dyspnea, lasting from 7 to 14 days and rapidly progressing in most patients to respiratory failure.

Diagnosis of acute interstitial pneumonia (Hammain-Rich syndrome)

Diagnosis of acute interstitial pneumonia (Hammen-Rich syndrome) is based on the analysis of the history of the patient, the results of radiation studies, lung function and histological examination of the biopsy material. Changes in radiography of chest organs are similar to those in ARDS and correspond to diffuse bilateral dimming of pulmonary fields. In HRCT, bilateral focal symmetrical darkening areas are identified as matte glass, sometimes bilateral foci of consolidation of air spaces, mainly in subpleural zones. An uninformed "cellular lung," which usually affects no more than 10% of its volume, may be noted. Standard laboratory studies are not informative.

The diagnosis of "acute interstitial pneumonia (Hammen-Rich syndrome)" is confirmed by biopsy results when diffuse damage to alveoli is noted in the study of the material in the absence of known causes of ARDS and other possible causes of diffuse alveolar lesions (eg, sepsis, drug use, intoxication, exposure and viral infections ). Acute interstitial pneumonia (Hammen-Rich syndrome) is differentiated with diffuse alveolar hemorrhagic syndrome, acute eosinophilic pneumonia and idiopathic obliterating bronchiolitis with organizing pneumonia.

trusted-source[4], [5], [6]

Treatment of acute interstitial pneumonia (Hammain-Rich syndrome)

Treatment of acute interstitial pneumonia (Hammen-Rich syndrome) is supportive and usually requires artificial ventilation. Glucocorticoid therapy is usually used, but its effectiveness has not been established.

What is the prognosis of acute interstitial pneumonia (Hammain-Rich syndrome)?

Acute interstitial pneumonia (Hammain-Rich syndrome) has an unfavorable prognosis. Mortality is more than 60%; most patients die within 6 months of the onset of the disease, the cause of death is usually respiratory failure. In patients who survive after the initial acute episode of the disease, complete recovery of lung function occurs, although relapses are possible.

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