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Acute interstitial pneumonia (Hammen-Rich syndrome)
Last reviewed: 05.07.2025
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[ What causes acute interstitial pneumonia (Hamman-Rich syndrome)?
Acute interstitial pneumonia is differentiated histologically by the presence of organized diffuse alveolar damage and nonspecific changes that also occur in other types of lung injury not associated with idiopathic interstitial pneumonia. The sign of organized diffuse alveolar damage is widespread pronounced edema of the alveolar septa with infiltration by inflammatory cells; proliferation of fibroblasts; the presence of single hyaline membranes and their thickening. The septa are lined with atypical, hyperplastic type II pneumocytes, and the air spaces are collapsed. Thrombi that are not specific form in small arteries.
Symptoms of acute interstitial pneumonia (Hamman-Rich syndrome)
Acute interstitial pneumonia (Hamman-Rich syndrome) is characterized by the sudden development of fever, cough, and shortness of breath, lasting 7 to 14 days and rapidly progressing to respiratory failure in most patients.
Diagnosis of acute interstitial pneumonia (Hamman-Rich syndrome)
Diagnosis of acute interstitial pneumonia (Hamman-Rich syndrome) is based on the analysis of anamnesis data, results of radiological studies, pulmonary function tests and histological examination of biopsy material. Changes in chest radiography are similar to those in ARDS and correspond to diffuse bilateral opacities of the lung fields. HRCT reveals bilateral focal symmetrical areas of ground-glass opacities, sometimes - bilateral foci of consolidation of air spaces, mainly in subpleural zones. Formation of an indistinct "honeycomb lung" may be noted, usually affecting no more than 10% of its volume. Standard laboratory studies are not informative.
The diagnosis of acute interstitial pneumonia (Hamman-Rich syndrome) is confirmed by biopsy when diffuse alveolar damage is noted in the absence of known causes of ARDS and other possible causes of diffuse alveolar damage (eg, sepsis, drug use, intoxication, radiation, and viral infections). Acute interstitial pneumonia (Hamman-Rich syndrome) is differentiated from diffuse alveolar hemorrhagic syndrome, acute eosinophilic pneumonia, and idiopathic obliterating bronchiolitis with organizing pneumonia.
Treatment of acute interstitial pneumonia (Hamman-Rich syndrome)
Treatment of acute interstitial pneumonia (Hamman-Rich syndrome) is supportive and usually requires mechanical ventilation. Glucocorticoid therapy is commonly used, but its effectiveness has not been established.
What is the prognosis for acute interstitial pneumonia (Hamman-Rich syndrome)?
Acute interstitial pneumonia (Hamman-Rich syndrome) has a poor prognosis. Mortality is over 60%; most patients die within 6 months of onset, usually from respiratory failure. Patients who survive the initial acute episode of the disease have complete recovery of lung function, although relapses are possible.