Abstract
Granulomatous–lymphocytic interstitial lung disease (GLILD) has been defined as “a distinct clinico-radio-pathological ILD [interstitial lung disease] occurring in patients with CVID [common variable immunodeficiency disorders], associated with a lymphocytic infiltrate and/or granuloma in the lung, and in whom other conditions have been considered and where possible excluded… usually seen in the context of multisystem granulomatous/inflammatory involvement” [1]. The immune and inflammatory complications of CVID such as GLILD are important and associated with reduced survival [2]. However, as a rare manifestation of a rare disease, the scientific understanding and evidence basis to inform effective diagnosis and management of GLILD [3] are limited. There are challenges with the definition of GLILD presented above and the terminology more widely of ILD in people with CVID, which requires further consensus. In this manuscript, we use the term GLILD to describe the heterogeneous ILD seen as part of multisystem immune dysregulation in a substantial minority of people with CVID. A recent international survey of clinicians found little uniformity in diagnostic and therapeutic interventions, identifying an urgent need for new evidence to support consensus guidance [4]. In 2019, the European Respiratory Society (ERS) established a Clinical Research Collaboration to address GLILD (eGLILDnet) [5]. eGLILDnet aims to promote the exchange of research ideas among clinicians and scientists in order to plan, conduct, evaluate and publish clinical and translational studies. Better evidence to diagnose and manage GLILD requires new multicentre research and to this end, we have conducted and here report the results of an international research prioritisation exercise in GLILD. This was a partnership between multiprofessional clinicians and people living with GLILD.
The first ever research prioritisation exercise in GLILD: this survey identified areas of interest in the diagnosis, treatment and management of GLILD, which can be used as a roadmap for future research
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