Bintalib, HM; Burns, SO; Hurst, JR; (2023) Granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency. ERS Monograph , 2023 (100) pp. 310-319. 10.1183/2312508X.10019222.

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Abstract

Granulomatous and lymphocytic ILD (GLILD) is a rare noninfectious complication affecting patients with common variable immunodeficiency disorder (CVID). Although the pathophysiology is not fully understood, it is likely to involve autoimmune dysregulation. GLILD usually occurs together with other noninfectious complications, raising morbidity and mortality. The symptoms are cough and dyspnoea, but some patients remain asymptomatic. The clinical picture includes nodules, interlobular septal thickening, ground-glass opacities and splenomegaly on imaging. Lung function varies among patients but is typically restrictive with decreased gas transfer. Excluding malignancy and infection is necessary as these may cause similar clinical presentations. Lung biopsy can be performed to confirm the diagnosis. GLILD usually appears as mixed histological patterns, including lymphoid interstitial pneumonitis, follicular bronchiolitis, lymphoid hyperplasia, organising pneumonia and non-necrotising granuloma. Optimising immunoglobulin replacement therapy is essential before initiating treatment. Glucocorticoids remain the recommended first-line therapy; however, relapse usually occurs on dose tapering, and second-line immunosuppressants are recommended.

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