Primary Immunodeficiency Disease (PIDD) (#ACAAI19 Twitter summary)

Dr Alexei Gonzalez @alexeigonzmd: ESID data: between 5-66% of patients with CVID will develop Lund disease; 1/4 will have ILD.

GLILD has three pathologic patterns: granulomas, lymphocytic interstitial pneumonitis, and follicular bronchiolitis. GLILD future treatment: RTX x 4 cycles followed by 18 months of AZA or MMF. GLILD: steroids are 50% ineffective - consider dual immunosuppressive therapy.

Sasha Alvarado, DO @sashaaalvarado: Elena Perez, MD: 29% of patients with CVID have autoimmune manifestations.

Lisa Forbes, MD: Whole exome sequencing is preferred, but commercial labs offer accessible PID panels, and there are many experts who are happy to collaborate with private practitioners on variants of unknown significance.