Granulomatous lymphocytic interstitial lung disease: Description of a series of 9 cases

Abstract

Abstract Introduction Granulomatous-lymphocytic interstitial lung disease (GLILD) is one of the most serious non-infectious complications in patients with common variable immunodeficiency (CVID). Its diagnosis and treatment are challenging. Objective To analyse the characteristics of Hospital General Universitario de Alicante patients with CVID and GLILD. Material and methods Descriptive study of patients with CVID and GLILD diagnosed from 2000 to 2020. Results Of the 42 patients with CVID, 9 had GLILD (21%). Mean age at diagnosis of 39 years. Sixty-six percent of the CVID was type MB0. Fifty-five percent had decreased BLs. There was a decrease in DLCO by 89%. Surgical lung biopsy (SLB) was performed in 78%. The most frequent extrapulmonary manifestation was adenopathy (78%). One patient had a heterozygous pathological mutation in the CTLA4 gene. Of the patients, 67% received combined corticosteroid treatment with Rituximab. Conclusions GLILD is a rare complication of CVID whose diagnosis and treatment are a challenge. Its diagnosis requires a high index of suspicion, therefore a multidisciplinary diagnostic approach and combined treatment could provide a good result in the adult population.