[Three autopsy cases of chronic necrotizing pulmonary aspergillosis]

Chronic necrotizing pulmonary aspergillosis (CNPA), also called semi-invasive pulmonary aspergillosis, was first described in the early 1980s as a distinct type of pulmonary aspergillosis. CNPA was an indolent, cavitary, infectious process of the lung parenchyma secondary to local invasion by Aspergillus species. Diagnosis is confirmed by pathological evidence of lung tissue invasion by the fungus. Clinical diagnosis by combined clinical, radiological and laboratory findings is needed because histopathological confirmation cannot always be obtained in the clinical setting. CNPA is recognized as a clinical syndrome in Japan, and has been poorly defined histologically. We report three autopsy cases of CNPA evaluated histopathologically. Subjects were middle-aged to older men with a medical history of pulmonary mycobacterial infection who presented with pulmonary or systemic symptoms. Radiologically, progressive upper lobe cavitary infiltrates were seen with mycetomas and infiltration in lower lung fields. Clinically, CNPA was diagnosed based on 2007 Japanese guidelines for the diagnosis and treatment of deep fungal infection. Subjects died of respiratory failure within one month to three years of diagnosis despite antifungal therapy including micafungin, voriconazole, or itraconazole combined with broad spectrum antibiotics. Autopsy findings showed cavities containing the fungus but no fungal invasion of viable lung tissue. The area of progressive infiltration revealed bacterial pneumonia, organizing pneumonia or organizing diffuse alveolar damage without Aspergillus. In conclusion, CNPA is a chronic progressive clinical form of pulmonary aspergillosis with significant morbidity and mortality.