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Granulomatous-Lymphocytic Interstitial Lung Disease in CVID

Imaging findings

CT chest shows multifocal, ill-defined subsegmental ground-glass opacities, consolidations with air bronchograms, and significant mediastinal/hilar lymphadenopathy. Serial imaging over a year shows spontaneous waxing and waning of the pulmonary opacities. The patient has a history of Evans syndrome and hypogammaglobulinemia.

Key takeaways

Granulomatous-lymphocytic interstitial lung disease (GLILD) is a rare pulmonary manifestation of common variable immunodeficiency (CVID). It is characterized by lymphoid hyperplasia and non-caseating granulomas, mimicking sarcoidosis. Key features include lymphadenopathy, waxing/waning ground-glass or consolidative lesions, and splenomegaly. Evans syndrome (autoimmune hemolytic anemia and thrombocytopenia) can co-exist with GLILD.

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