Allergy and Airway |

Better Late Than Never: A Late Presentation of Common Variable Immunodeficiency FREE TO VIEW

Susan Mucha, MD; Mark Lega, MD; Marvin Balaan, MD
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Allegheny General Hospital, Pittsburgh, PA

Chest. 2014;145(3_MeetingAbstracts):2A. doi:10.1378/chest.1826003
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SESSION TITLE: Asthma Case Report Posters

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM

INTRODUCTION: We present a 52 year old patient masquerading as a “difficult to control asthmatic”. Her ultimate diagnosis is common variable immunodeficiency complicated by recurrent pulmonary infections, follicular bronchitis, and organizing pneumonia. This diagnosis is rare, but should be entertained in our patient’s age group. The common variable immunodeficiencies (CVID) are relatively frequently encountered syndromes of various types of B-cell activation or differentiation defects, most often diagnosed during late childhood or early adult life. (1-4) Clinical manifestations of CVID include recurrent infections of the respiratory tract and, less frequently, autoimmune, granulomatous, or lymphoproliferative disorders.

CASE PRESENTATION: This is a 52 year old female asthmatic nonsmoker presenting with eleven years of recurrent pneumonias, asthma exacerbations and weight loss. She had persistent cough, fatigue, and dyspnea after a bout of pneumonia treated with azithromycin. Frustrated, she presented for a second opinion. Chest CT showed bilateral airspace disease and bronchiectasis predominantly in the lower lobes. Transbronchial biopsies revealed “possible” cryptogenic organizing pneumonia and moderate haemophilus influenza on culture. A workup was negative for connective tissue disease and HIV, but revealed profound IgG, IgA, and IgM deficiencies. Due to serial CT scans over the years showing interstitial changes, a VATS biopsy was done exhibiting follicular bronchitis, bronchiectasis, constrictive bronchiolitis, and acute organizing pneumonia. She has begun lifelong immunoglobulin replacement therapy and will soon begin corticosteroid therapy.

DISCUSSION: The age of onset for CVID is usually in the second to third decade of life. (6-9) Long-term outcome is significantly influenced by delay of diagnosis and treatment in the presence of chronic inflammatory complications. (6) Our patient did not come to diagnosis until age 52, despite multiple physician encounters over many years.

CONCLUSIONS: CVID should be considered in adults of any age when presenting with recurrent pneumonias and bronchospasm, as this could significantly improve outcomes if diagnosed before chronic lung changes occur. This case reminds us of the old adage that “all that wheezes is not asthma!”

Reference #1: Murray JF, Nadel JA: Textbook of Respiratory Medicine: 5th Edition. Saunder, an imprint of Elsevier, Philadelphia, 2010.

Reference #2: Hermans, PE, Diaz-Buxo, JA, Stobo JO: Idiopathic late-onset immunoglobulin deficiency. Clinical observations in 50 patients. Am J Med. 61: 221-237, 1976.

Reference #3: Cunningham-Rundles C: Clinical and immunological analysis of 150 patients with common variable immunodeficiency. J Clin Immunol. 9:22-33, 1989.

DISCLOSURE: The following authors have nothing to disclose: Susan Mucha, Mark Lega, Marvin Balaan

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