Common variable immunodeficiency

Section of Allergy and Immunology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
American Journal of Rhinology and Allergy (Impact Factor: 1.81). 07/2013; 27(4):260-5. DOI: 10.2500/ajra.2013.27.3899
Source: PubMed

ABSTRACT Common variable immunodeficiency (CVID) is a common primary immunodeficiency characterized by a failure in B-cell differentiation with defective immunoglobulin production. Affected patients are uniquely susceptible to recurrent infection with encapsulated organisms and have an increased propensity for the development of inflammatory and autoimmune manifestations. The diagnosis of CVID is commonly delayed and the underlying cause of the disorder is not understood. Replacement antibody therapy reduces the risk of serious infections. However, optimal treatment regimens for the uncommon manifestations associated with this disease, such as granulomatous lymphocytic interstitial lung disease, require further research.

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Available from: Jonathan S Tam, Dec 09, 2014
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    • "Regarding the principal differential diagnosis in our case, significant causes of adult onset immune dysfunction that had to be excluded were idiopathic CD4 lymphocytopenia (ICL) and adenosine deaminase (ADA) deficiency. Secondary etiologies associated with hypogammaglobulinemia, such as lymphoma, leukemia, thymoma, sarcoidosis, nephrotic syndrome, protein-losing enteropathy, and intestinal lymphangiectasia, were excluded during investigation [3, 6]. "
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    ABSTRACT: Late onset combined immunodeficiency (LOCID) is a recently described variant of common variable immunodeficiency (CVID), involving adult patients presenting with opportunistic infections and/or low CD4+ lymphocyte counts. A 36-year-old male with unremarkable past medical history presented with fever, respiratory failure, and lymphocytopenia. He was found to have Pneumocystis jiroveci pneumonia (PJP), subsequently complicated by recurrent hospital-acquired Pseudomonas aeruginosa pneumonia and immune reconstitution phenomena, attributed to restoration of immunoglobulin levels. Clinicians should be aware of LOCID, which could be confused with HIV infection/AIDS or idiopathic CD4 lymphocytopenia. In the English bibliography there is only one case report, where PJP was the initial presentation of CVID (that case would probably be classified as LOCID). Phenomena of immune reconstitution are described in various settings, including primary immunodeficiency, manifesting as temporary clinical and radiologic deterioration and leading to misperceptions of therapeutic failure and/or presence of alternative/additional diagnoses.
    Case Reports in Medicine 03/2014; 2014:801805. DOI:10.1155/2014/801805