(Accelerated interstitial pneumonia; Hamman-Rich syndrome)
The acute interstitial pneumonia (AIP) is an idiopathic version of the acute respiratory distress syndrome (ARDS).
AIP, a form of idiopathic interstitial pneumonia, equally affects apparently healthy men and women, usually> 40 years.
The acute interstitial pneumonia (AIP) is an idiopathic version of the acute respiratory distress syndrome (ARDS). AIP, a form of idiopathic interstitial pneumonia, equally affects apparently healthy men and women, usually> 40 years. The AIP is histologically characterized by a diffuse alveolar damage organizing itself, a non-specific finding, which also occurs in other unrelated with idiopathic interstitial pneumonia causes pathological changes in the lungs. The main features of an organizing diffuse Alevolarschadens are a diffuse significant edema of the alveolar walls with infiltrating inflammatory cells, fibroblast proliferation, occasionally hyaline membranes and thickening of the alveolar walls. The septa are lined with atypical, hyperplastic type II pneumocytes and collapses the air spaces. In small arteries nonspecific thrombi develop. The symptoms consist of the sudden onset of fever, cough and shortness of breath, the increase in most patients in severity over 7-14 days, and progress to respiratory arrest. Diagnosis High-resolution CT (HRCT) Usually lung biopsy The diagnosis is made (diffuse bilateral shading of the air spaces such. B.) in patients with symptoms, discomfort and chest x-ray findings of ARDS. The diagnosis is made by high-resolution CT (HRCT), but usually requires biopsy. HRCT in both sides mottled symmetrical milk glass clouding and sometimes bilateral areas with consolidation of the air spaces in predominantly pleura distribution are seen. One observes a slight alveolar reconstruction, which usually affects <10% of the lung. Routine laboratory tests are nonspecific and lead generally not on. The diagnosis is confirmed by surgical lung biopsy, diffuse alveolar damage with no known causes of ARDS and diffuse alveolar damage shows (for example, sepsis, drugs, toxins, radiation, viral infection). Acute exacerbation of underlying lung disease (especially acute exacerbation of idiopathic pulmonary fibrosis) must be considered and, some cases that were previously characterized as AIP explain. A biopsy is often necessary to distinguish AIP from a diffuse alveolar Hämorrhagiesyndrom, acute eosinophilic pneumonia and cryptogenic organizing pneumonia. Supportive therapy Treatment is supportive and usually requires mechanical ventilation, often using the same methods as with ARDS (including low Hubvolumenbeatmung). Generally, also corticosteroids are used, their effectiveness is not proven. The mortality rate is> 60%; most patients die within 6 months after initial presentation; Cause of death is usually an acute lung failure. In patients who survive the initial acute episode, a complete restoration of lung function can be performed, although relapses can occur.