Abstract
Pulmonary abnormalities are present in as many as 60% of patients with a primary humoral immunodeficiency, and chronic changes and recurrent infections in the respiratory tract are the main causes of morbidity and mortality among those patients.
Humoral immunodeficiencies, which are characterized by defective production of antibodies, are the most common types of primary immunodeficiency. Pulmonary changes are present in as many as 60% of patients with humoral immunodeficiency. Chronic changes and recurrent infections in the respiratory airways are the main causes of morbidity and mortality in those affected by a humoral immunodeficiency. Medical imaging, especially computed tomography (CT), plays a crucial role in the initial detection and characterization of changes and in monitoring the response to therapy. The spectrum of abnormalities seen at thoracic imaging includes noninfectious airway disorders, infections, chronic lung diseases, chronic inflammatory conditions (granulomatosis, interstitial pneumonias), and benign and malignant neoplasms. Recognition of characteristic CT and radiographic features, and correlation of those features with clinical and laboratory findings, are necessary to differentiate between the many possible causes of parenchymal and mediastinal disease seen in patients with primary humoral immunodeficiencies.
References
- 1 . Pulmonary and sinusal changes in 45 patients with primary immunodeficiencies: computed tomography evaluation. J Comput Assist Tomogr 2007;31:620–628. Crossref, Medline, Google Scholar
- 2 . Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol 1999;92: 34–48. Crossref, Medline, Google Scholar
- 3 . Agammaglobulinemia. Pediatrics 1952; 9:722–728. Crossref, Medline, Google Scholar
- 4 . Primary cellular immunodeficiencies. J Allergy Clin Immunol 2002;109:747–757. Crossref, Medline, Google Scholar
- 5 . Common variable immunodeficiency. Curr Allergy Asthma Rep 2001;1: 421–429. Crossref, Medline, Google Scholar
- 6 . Infections in 252 patients with common variable immunodeficiency. Clin Infect Dis 2008;46:1547–1554. Crossref, Medline, Google Scholar
- 7 . Inflammatory and autoimmune complications of common variable immune deficiency. Autoimmun Rev 2006;5: 156–159. Crossref, Medline, Google Scholar
- 8 . Chest high resolution CT in adults with primary humoral immunodeficiency. Br J Radiol 1996;69:1108–1116. Crossref, Medline, Google Scholar
- 9 . Respiratory disorders in common variable immunodeficiency. Respir Med 2001;95:191–195. Crossref, Medline, Google Scholar
- 10 . Respiratory manifestations of common variable immunodeficiency in adults [in French]. Rev Mal Respir 2003;20: 126–133. Medline, Google Scholar
- 11 . Radiologic findings of adult primary immunodeficiency disorders: contribution of CT. Chest 1994;106:490–495. Crossref, Medline, Google Scholar
- 12 . Airway obstruction in adults with recurrent respiratory infections and IgG deficiency. Chest 1994;105:1066–1072. Crossref, Medline, Google Scholar
- 13 . Characterization of Haemophilus influenzae isolates from the respiratory tract of patients with primary antibody deficiencies: evidence for persistent colonizations. Scand J Infect Dis 1995;27:303–313. Crossref, Medline, Google Scholar
- 14 . Clinical and immunologic studies of common variable immunodeficiency. Curr Opin Pediatr 1994;6:676–681. Crossref, Medline, Google Scholar
- 15 . Granulomatous disease in common variable immunodeficiency. Ann Intern Med 1997;127:613–617. Crossref, Medline, Google Scholar
- 16 . Sarcoidosis and common variable immunodeficiency: report of 8 cases and review of the literature. Medicine (Baltimore) 1996;75:251–261. Crossref, Medline, Google Scholar
- 17 . Pulmonary interstitial disease in Ig deficiency. Chest 2002;122:1594–1603. Crossref, Medline, Google Scholar
- 18 . Organizing pneumonia related to common variable immunodeficiency: case report and literature review. Respiration 2000;67:467–470. Crossref, Medline, Google Scholar
- 19 . Lung diseases in patients with common variable immunodeficiency: chest radiographic, and computed tomographic findings. J Comput Assist Tomogr 2006;30:828–838. Crossref, Medline, Google Scholar
- 20 . What every radiologist should know about idiopathic interstitial pneumonias. RadioGraphics 2007;27:595–615. Link, Google Scholar
- 21 . Lymphocytic interstitial pneumonia associated with common variable immunodeficiency resolved with intravenous immunoglobulins. Thorax 2006;61:1096–1097. Crossref, Medline, Google Scholar
- 22 . Common variable hypogammaglobulinemia in children: clinical and immunologic observations in 30 patients. Am J Dis Child 1983;137:833–837. Crossref, Medline, Google Scholar
- 23 . Nodular lymphoid hyperplasia in the lung. Thorac Cardiovasc Surg 1996;44:210–212. Crossref, Medline, Google Scholar
- 24 . Multifocal nodular lymphoid hyperplasia of the lung. J Thorac Imaging 2005;20:239–241. Crossref, Medline, Google Scholar
- 25 . Nodular lymphoid hyperplasia of the lung: a clinicopathologic study of 14 cases. Am J Surg Pathol 2000;24:587–597. Crossref, Medline, Google Scholar
- 26 . Mediastinal lymph node enlargement and splenomegaly in primary hypogammaglobulinaemia. Clin Radiol 1995;50:489–491. Crossref, Medline, Google Scholar
- 27 . Lymphoma in common variable immunodeficiency: interplay between immune dysregulation, infection and genetics. Curr Opin Hematol 2008;15:368–374. Crossref, Medline, Google Scholar
- 28 . Non-Hodgkin lymphoma in common variable immunodeficiency. Am J Hematol 1991;37: 69–74. Crossref, Medline, Google Scholar
- 29 . Thymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of the literature. Ann Allergy Asthma Immunol 2007;98:185–190. Crossref, Medline, Google Scholar
Article History
Received: Mar 23 2009Revision received: Apr 21 2009
Revision received: May 20 2009
Accepted: May 20 2009
Published in print: Nov 2009