British Lung Foundation/United Kingdom Primary Immunodeficiency Network Consensus Statement on the Definition, Diagnosis, and Management of Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders

A proportion of people living with common variable\ud immunodeficiency disorders develop granulomatouslymphocytic\ud interstitial lung disease (GLILD). We aimed to\ud develop a consensus statement on the definition, diagnosis, and\ud management of GLILD. All UK specialist centers were contacted\ud and relevant physicians were invited to take part in a 3-round\ud online Delphi process. Responses were graded as Strongly Agree,\ud Tend to Agree, Neither Agree nor Disagree, Tend to Disagree,\ud and Strongly Disagree, scored D1, D0.5, 0, L0.5, and L1,\ud respectively. Agreement was defined as greater than or equal to\ud 80% consensus. Scores are reported as mean – SD. There was\ud 100% agreement (score, 0.92 – 0.19) for the following\ud definition: “GLILD is a distinct clinico-radio-pathological ILD\ud occurring in patients with [common variable immunodeficiency\ud disorders], associated with a lymphocytic infiltrate and/or\ud granuloma in the lung, and in whom other conditions have been\ud considered and where possible excluded.” There was consensus\ud that the workup of suspected GLILD requires chest computed\ud tomography (CT) (0.98 – 0.01), lung function tests (eg, gas\ud transfer, 0.94 – 0.17), bronchoscopy to exclude infection (0.63 –\ud 0.50), and lung biopsy (0.58 – 0.40). There was no consensus on\ud whether expectant management following optimization of\ud immunoglobulin therapy was acceptable: 67% agreed, 25%\ud disagreed, score 0.38 – 0.59; 90% agreed that when treatment\ud was required, first-line treatment should be with corticosteroids\ud alone (score, 0.55 – 0.51).