Case Challenge # 15 presented a 42-year-old white female with recurrent pneumonia in the setting of chronic dyspnea x 15 years without a determined etiology. She also has a history of COPD without smoking and recurrent sinus infections since childhood. She also notes chronic loose stools with occasional constipation for 10 years. Her mother also had COPD and recurrent sinus infections. Exam reveals no clubbing or nasal polyps. CT chest reveals bronchial wall thickening and dilatation predominantly in the lower lung fields consistent with bronchiectasis.
Which of the following is the best initial test to establish the diagnosis?
The correct answer is measurement of immunoglobulin levels!
The patient has CVID (common variable immunodeficiency)
- CVID is a heterogeneous disorder characterized by markedly reduced serum concentrations of IgG, low levels of IgA and/or IgM, and poor or absent responses to immunization.
- The disorder affects males and females equally, and usually has a later age of onset than other antibody-deficiency disorders (i.e., > 10 years of age).
- It is associated with recurrent sinopulmonary infections, autoimmune and granulomatous disease, gastrointestinal complications and an enhanced risk of malignancy (e.g., lymphoma and gastric carcinoma).
- Some patients may also present with bronchiectasis (irreversible widening of portions of the bronchi resulting from damage to the airway wall), which is a common cause of morbidity and mortality in these patients.
- Ig replacement therapy is the mainstay of therapy for antibody-deficiency disorder
- Antibiotic and antifungal prophylaxis are also recommended for some to prevent the frequency and severity of infections.
Case challenge #16 (aka the Parkland Files!) will be posted next week!
Allergy, Asthma & Clinical Immunology 2011, 7(Suppl 1):S11 . Published under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0).