Abstract

Chronic pulmonary aspergillosis (CPA) is a chronic fungal infection of the lung associated with high morbidity and mortality. The CPA Research network (CPAnet) registry established in 2018 is an international multicenter collaboration aiming to improve CPA knowledge and patient care. This study’s aim was to describe the data collection process and content of CPAnet registry with preliminary clinical data. In the CPAnet registry, clinical data are collected through a web-based questionnaire. Data include CPA phenotype, comorbidities, treatment, outcome, and follow-up from several international centers. An exemplary descriptive analysis was performed on 74 patients, who were registered online before April 2020. CPA patients were predominantly (72%) male, 39% had chronic obstructive pulmonary disease, and 68% had a history of smoking. Chronic cavitary pulmonary aspergillosis was the most common CPA subtype (62%). In 32 patients (52%), voriconazole was the preferred first-line therapy. The multicenter multinational CPAnet registry is a valuable approach to gather comprehensive data on a large study population and reflects real-world clinical practice rather than focusing on specific patient populations in more specialized centers. Additional CPA reference centers are being encouraged to join this promising clinical research collaboration.

Highlights

  • Chronic pulmonary aspergillosis (CPA) is a disease spectrum consisting of different phenotypes of severe chronic fungal infection of the lung [1,2]

  • Voriconazole was the preferred first-line treatment (52%), followed by itraconazole (42%). This is congruent with other studies and the current guidelines, where both are recommended as first-line treatments [3,17,19]

  • As the registry is not restricted to certain centers or countries, there is a great potential of exploring, amongst others, the incidence and prevalence over time, referral patterns for CPA centers, risk factors of CPA development, and predictors for disease severity and mortality

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Summary

Introduction

Chronic pulmonary aspergillosis (CPA) is a disease spectrum consisting of different phenotypes of severe chronic fungal infection of the lung [1,2]. CPA patients are hampered by a substantial morbidity affecting approximately 3 million people worldwide and with an overall 5-year mortality of up to 80% that estimates 450,000 annual deaths [3,4,5]. Despite this apparently high disease burden, paradoxically CPA prevalence seems to be low in single centers [6]. Chronic obstructive pulmonary disease (COPD), prior tuberculosis (TB), non-tuberculous mycobacterial (NTM)

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