Pulmonary Disease in Primary Immunodeficiency Disorders

Pulmonary complications are an extremely common manifestation of primary immunodeficiency disorders (PIDD). Infections are the most common abnormality and can lead to specific pulmonary complications. Patients with PIDD can present with a variety of manifestations such as recurrent bacterial, viral, or fungal pneumonia, bronchiectasis, granulomatous disease, and obstructive or restrictive lung disease. Some of the PIDDs can have unique lung abnormalities seen with specific defects. This review covers the respiratory manifestations seen in patients with a variety of primary immunodeficiencies. This recognition is important to reduce morbidity and mortality in these patients and possibly prevent the occurrence of chronic lung damage.