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.
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