Acute Interstitial Pneumonia–Hamman-Rich Syndrome: Clinical Characteristics and Diagnostic and Therapeutic Considerations

Abstract

Acute interstitial pneumonia (Hamman-Rich syndrome) is an idiopathic, rapidly progressive and, at times, fatal form of interstitial lung disease. A transbronchial biopsy is a logical first diagnostic step, to be followed by an open lung biopsy, if necessary. Response to corticosteroids in our series was minimal. In patients who fail to respond to conventional therapy and are otherwise appropriate candidates, lung transplantation may be considered as an additional alternative.