Granulomatous-lymphocytic interstitial lung disease (GLILD) in CVID: A case-control single center retrospective study

8 to 22% of patients with Common Variable Immunodeficiency (CVID) develop GLILD, associated with bad prognosis. GLILD is characterized by diffuse radiological abnormalities and/or histologic evidence of granulomatous inflammation with lymphoproliferative changes. Proper management is unknown. In this retrospective single-centre study, chest HRCT scans of 34 CVID patients were blind-reviewed by 2 radiologists. 15/34 patients presented HRCT findings consistent with GLILD. Different parameters were compared between patients with HRCT patterns consistent with GLILD and those without GLILD features. The mean percentage of circulating B lymphocytes was lower in GLILD-patients. GLILD-patients presented a reduced percentage of switched-memory B cells and a higher percentage of activated B cells. IgG, IgA and IgM levels at diagnosis were lower in GLILD patients, requiring higher levels of IgG replacement. Splenomegaly was found in 53.3% of GLILD patients and in 15% of controls. 3/15 GLILDpatients underwent splenectomy due to autoimmune cytopenias. None of the controls had autoimmune cytopenia. 14/15 GLILD-patients had FEV1 and FVC > 70%. DLCO was reduced (<70%) in 5/15 patients and significantly lower in GLILD-patients than in controls. 8/15 GLILD-patients underwent bronchoscopy. 6/8 presented BALF lymphocytosis, with an increased CD19+ fraction in 3 (mainly activated B cells) and an increased CD4/CD8 ratio in 2 patients. GLILD is a pulmonary manifestation of a multisystemic lymphoproliferative disorder with evidence of immune dysregulation. GLILD HRCT features should always be sought in CVID patients. Specific immunological parameters might help the therapeutic approach.