Interstitial lung disease in CVID (GLILD): Clinical presentation and comparison to CVID without ILD

Introduction: Common variable immunodeficiency (CVID) patients are susceptible to respiratory diseases including bronchiectasis and granulomatous lymphocytic interstitial lung disease (GLILD). As data on evaluating CVID with and without GLILD are sparse, we aimed to compare these groups. Methods: 21 CVID patients were analyzed retrospectively for characteristics and outcomes and grouped into CVID with (ILD,n=9) and without GLILD (noILD,n=12). CT scoring (CTS) assessed 11 patterns (ground glass opacities, reticulations, traction bronchiectasis, honeycombing, consolidations, air-trapping, nodules, bronchial wall thickening, bronchiectasis, mucus plugging, effusion). Histology scoring (HS) evaluated changes of the bronchial wall and of the interstitium (fibrosis, inflammation, anthracosis, granuloma, tumor, infection, metaplasia, mucoid impactation, microhoneycombing, eosinophilia). Results: Baseline characteristics were similar between groups. Most CVID patients received immunoglobulins (Ig) and all ILD patients were treated immunosuppressive (IS). Under Ig and IS lung function remained stable in ILD. CTS were worse for ILD than noILD (ILD:16.5±8.7 vs. noILD:9.4±4.9, p=0.02) while HS (ILD:5.8±2.7, noILD 6.0±2.6) were similar. CTS deteriorated in ILD but not in noILD with pattern changes in ILD, from initial mainly GGO to fibrosis (p<0.01), while noILD, mainly bronchial wall thickening, remained stable. HS mainly showed fibrosis and chronic inflammation in both groups. After a median of 3.8 years, there were no deaths. Conclusion: CVID with and without GLILD has similar clinical characteristics but differs mainly in CT scoring. Future work has to assess clinical predictors of GLILD.