Evolution of IgG Subclass Deficiency into Common Variable Immunodeficiency in a Child with Pulmonary Interstitial Glycogenosis

We present a 5-year-old boy who initially presented during infancy with pulmonary interstitial glycogenosis (PIG) and recurrent sinopulmonary infections. At 3.5 years old, IgG-2 subclass deficiency associated with low IgA level and selective antibody deficiency was diagnosed. Subsequently, the immune deficiency evolved into common variable immunodeficiency (CVID). This is the first case reported of the association of PIG and CVID. In children with PIG who have recurrent infections, associated immunodeficiency should be suspected.