Follicular Bronchiolitis Associated With Primary IgG2/IgG4 Deficiency in a Previously Healthy 40-Year-Old Woman

Follicular bronchiolitis (FB) associated with immunodeficiency is not commonly reported in peer-reviewed literature. Herein, we present a case of FB associated with IgG2/IgG4 deficiency. The presence of non-specific respiratory symptoms, including cough and dyspnea with exertion, led to a CT scan of the chest, which showed diffuse, peripheral, micronodular tree-in-bud opacities and an isolated area of bronchiectasis. Despite an extensive workup, including a non-diagnostic transbronchial biopsy, no obvious etiology for the patient’s clinical presentation was established, and a surgical lung biopsy was needed to confirm the diagnosis of FB. Further lab testing to evaluate for immunodeficiency confirmed an isolated deficiency in both IgG2 and IgG4. Although treating the underlying cause of FB is the standard of care, there are no established guidelines regarding standard management of FB associated with immunodeficiency, specifically IgG2/IgG4 deficiency. Therefore, a careful evaluation for immunodeficiency should be considered when evaluating for the underlying etiology of FB, as management options differ depending on the underlying diagnosis.