Chronic fibrosing pulmonary aspergillosis: a cause of ‘destroyed lung’ syndrome

Abstract

Background: Chronic pulmonary aspergillosis (CPA) has substantial impact on quality of life. A subset of patients develops significant pulmonary fibrosis, identified either on biopsy or radiologically. The term chronic fibrosing pulmonary aspergillosis (CFPA) has been suggested.

Methods: We describe 11 patients with CFPA referred to our centre.

Results: Mean age was 58.5 years and five were male. In nine, fibrosis was already evident on presentation, while in two it developed 3 and 6 years later. The predominant radiological feature was extensive or complete involvement of the entire lung, with minimal contralateral involvement. All patients received prolonged antifungal treatment. Two patients had surgical treatment; both developed post-operative complications. The contralateral lung remained free of significant disease in all but three patients.

Conclusions: CFPA is a rare complication of CPA that is usually evident on presentation, but may develop after years in patients not on antifungals. Fibrosis resembles the ‘destroyed lung’ syndrome described after treated tuberculosis.

Keywords:

• Chronic pulmonary aspergillosis
• fibrosis
• destroyed lung
• outcome
• case series
• radiology

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.