Chest Infections |

Common Variable Immunodeficiency and Bronchiectasis: An Easily Missed Common Association FREE TO VIEW

Khalid Sherani, MD; Hineshkumar Upadhyay, MD; Abhay Vakil, MD; Kelly Cervellione, PhD; Craig Thurm, MD
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Jamaica Hospital Medical Center, Jamaica, NY

Chest. 2014;145(3_MeetingAbstracts):123A. doi:10.1378/chest.1826513
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SESSION TITLE: Infectious Disease Case Report Posters I

SESSION TYPE: Case Report Poster

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

INTRODUCTION: The etiology of bronchiectasis is varied with abnormal host defenses being one of the causes. We report the case of a 49-year-old male presenting with recurrent pneumonias. Imaging studies revealed bronchiectasis. Work up for bronchiectasis showed low immunoglobulin levels consistent with common variable immunodeficiency (CVID). The aim of this case report is to heighten awareness among physicians for the possibility of immunoglobulin deficiency in patients presenting especially with bronchiectasis, recurrent infections and sinus disease.

CASE PRESENTATION: A 49-year-old nonsmoker male presented with fever and productive cough for 1 week. He had multiple similar episodes in the past treated with systemic antibiotic therapy at another hospital. He also reported having recurrent sinusitis and was being considered for sinus surgery by ENT physician. As a child he reported having recurrent ear infections leading to chronic hearing loss. He denied any significant family history and had 3 healthy children. Physical exam revealed bronchial breath sounds in left lower lobe. Review of old radiologic studies revealed progressively worsening bronchiectasis predominantly involving left lower lobe (fig 1a), tree-in-bud opacities with loss of volume on the left (fig 2) and mediastinal adenopathy (fig 1b). In view of history and presentation immunoglobulin levels were sent. These showed IgG < 270 mg/dl, IgA < 40 mg/dl and Ig M < 25 mg/dl - consistent with CVID. Immunoglobulin therapy was started preventing further recurrence of infections and sinusitis in this patient.

DISCUSSION: Patients presenting with bronchiectasis, recurrent infections and sinus disease should be considered for immunoglobulin deficiency, ciliary disorders and cystic fibrosis. As demonstrated in this patient, CVID is known to be associated with bronchiectasis (11 %). It is also known to be associated with granulomatous and lymphocytic infiltrative diseases, sarcoidosis, follicular bronchiolitis, lymphoid hyperplasia, lymphoid interstitial pneumonia and lymphoma. The proposed mechanisms for the cause of bronchiectasis in CVID include recurrent infections and unregulated inflammation. In CVID, immunoglobulin replacement may slow progression of lung disease, although this has not been conclusively established.

CONCLUSIONS: Among the various etiologies of bronchiectasis, the potentially treatable ones like CVID should never be missed. Appropriate and early institution of treatment can potentially improve survival, prevent deterioration of lung function and improve quality of life.

Reference #1: Busse PJ, Farzan S, Cunningham-Rundles C, "Pulmonary complications of common variable immunodeficiency" Ann Allergy Asthma Immunol. 2007;98(1):1

Reference #2: Park JH, Levinson AI,"Granulomatous-lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID)" Clin Immunol. 2010;134(2):97

DISCLOSURE: The following authors have nothing to disclose: Khalid Sherani, Hineshkumar Upadhyay, Abhay Vakil, Kelly Cervellione, Craig Thurm

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