RT Journal Article SR Electronic T1 The broad spectrum of lung diseases in primary antibody deficiencies JF European Respiratory Review JO EUROPEAN RESPIRATORY REVIEW FD European Respiratory Society SP 180019 DO 10.1183/16000617.0019-2018 VO 27 IS 149 A1 Francesco Cinetto A1 Riccardo Scarpa A1 Marcello Rattazzi A1 Carlo Agostini YR 2018 UL http://err.ersjournals.com/content/27/149/180019.abstract AB Human primary immunodeficiency diseases (PIDs) represent a heterogeneous group of more than 350 disorders. They are rare diseases, but their global incidence is more relevant than generally thought. The underlying defect may involve different branches of the innate and/or adaptive immune response. Thus, the clinical picture may range from severe phenotypes characterised by a broad spectrum of infections to milder infectious phenotypes due to more selective (and frequent) immune defects. Moreover, infections may not be the main clinical features in some PIDs that might present with autoimmunity, auto-inflammation and/or cancer. Primary antibody deficiencies (PADs) represent a small percentage of the known PIDs but they are the most frequently diagnosed, particularly in adulthood. Common variable immunodeficiency (CVID) is the most prevalent symptomatic PAD.PAD patients share a significant susceptibility to respiratory diseases that represent a relevant cause of morbidity and mortality. Pulmonary complications include acute and chronic infection-related diseases, such as pneumonia and bronchiectasis. They also include immune-mediated interstitial lung diseases, such as granulomatous-lymphocytic interstitial lung disease (GLILD) and cancer. Herein we will discuss the main pulmonary manifestations of PADs, the associated functional and imaging findings, and the relevant role of pulmonologists and chest radiologists in diagnosis and surveillance.The spectrum of lung complications in primary antibody deficiency ranges from asthma or COPD to extremely rare and specific ILDs. Early diagnosis of the underlying immune defect might significantly improve patients' lung disease, QoL and long-term prognosis. http://ow.ly/5cP230kZvOB