Chronic pulmonary aspergillosis complicating sarcoidosis

Abstract

Chronic pulmonary aspergillosis (CPA) complicating sarcoidosis (SA) is associated with high mortality, and there is a lack of clarity regarding the relative contributions of SA or CPA.This was a retrospective single-centre study on CPA-SA.In total, 65 patients (44 men), aged 41.4±13.5 and 48.3±11.9 years at the time of SA and CPA diagnoses, respectively, were included between 1980 and 2015. Of these, 64 had fibrocystic SA, most often advanced, with composite physiological index (CPI) >40 (65% of patients) and pulmonary hypertension (PH) (31%), and 41 patients (63%) were treated for SA (corticosteroids or immunosuppressive drugs). Chronic cavitary pulmonary aspergillosis (CCPA) was the most frequent CPA pattern. Regarding treatment, 55 patients required long-term antifungals, 14 interventional radiology, 11 resection surgery and two transplantation. Nearly half of the patients (27; 41.5%) died (mean age 55.8 years); 73% of the patients achieved 5-year survival and 61% 10-year survival. Death most often resulted from advanced SA and rarely from haemoptysis. CPI, fibrosis extent and PH predicted survival. Comparison with paired healthy controls without CPA did not show any difference in survival, but a higher percentage of patients had high-risk mould exposure.CPA occurs in advanced pulmonary SA. CPA-SA is associated with high mortality due to the underlying advanced SA rather than to the CPA. CPI, fibrosis extent and PH best predict outcome.

Advanced Pulmonary Sarcoidosis

“…The best predictors of survival were CPI scoring, fibrosis extent and SAPH. 6 As previously noted, increased CPI and SAPH are factors predictive of increased mortality in advanced pulmonary sarcoidosis. 4,5 Diagnosis is made on the basis of imaging and is confirmed by direct evidence of Aspergillus or a positive galactomannan test on sputum and/or BAL samples, and a serological response to Aspergillus.…”

“…In the French study mentioned earlier, mycetomas were not associated with increased mortality. 6 The improved outcome for aspergillomas may be due to long term use of azoles, which have been shown in a randomized trial to improve outcome in pulmonary aspergillosis in sarcoidosis. 127…”

Radiographic and Histopathologic Features in Sarcoidosis: A Pictorial Display

“…Pulmonary arterial hypertension (PAH) may complicate sarcoidosis due to advanced fibrotic and cystic lung parenchymal involvement 6,49,[77][78][79][80] (►Fig. 42).…”

“…Chronic pulmonary aspergillosis (CPA, particularly mycetomas) may complicate advanced fibrocystic sarcoidosis. 49,78,[87][88][89][90][91][92][93][94][95][96] CT scans demonstrate a cavity with a well-defined mass, with or without an air crescent 97 (►Fig. 44).…”

“…One retrospective single-center study reported 65 patients with sarcoidosis who developed CPA between 1980 and 2015; all but one had fibrocystic sarcoidosis; 31% had PAH. 78 Treatment included long-term antifungals (n ¼ 55), interventional radiology (n ¼ 14), resectional surgery (n ¼ 11), and lung transplant (n ¼ 2). Overall mortality was 41.5%; 5-and 10-year survival rates were 73% and 61%, respectively.…”