Lung disease in primary antibody deficiency.

摘要: This Review summarises current knowledge on the pulmonary manifestations of primary antibody deficiency (PAD) syndromes in adults. We describe major PAD syndromes, with a particular focus common variable immunodeficiency (CVID). Respiratory infection is presenting feature syndromes. complications are frequent and responsible for much morbidity mortality associated these include acute infections, sequelae (eg, bronchiectasis), non-infectious immune-mediated (notably development granulomatous-lymphocytic interstitial lung disease CVID), an increased risk lymphoma. Although minor abnormalities detectable lungs most patients CVID by CT scanning, not all develop complications. Mechanisms maintenance health versus disease, bronchiectasis complications, now being dissected. review investigation, treatment, management strategies key research questions relating to both infectious lung.