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Diffuse Lung Disease |

Hypersplenism and Polyarthritis Are Strong Risk Factors for Common Variable Immunodeficiency Related Granulomatous-Lymphocytic Interstitial Lung Disease

Richard Hedelius, DO; Amar Mannina, BS; Joshua Solomon, MD; Jeffrey Swigris, DO; Evans Fernandez, MD
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Naval Medical Center San Diego, San Diego, CA


Chest. 2013;144(4_MeetingAbstracts):469A. doi:10.1378/chest.1704147
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Abstract

SESSION TITLE: Interstitial Lung Disease

SESSION TYPE: Original Investigation Slide

PRESENTED ON: Wednesday, October 30, 2013 at 02:45 PM - 04:15 PM

PURPOSE: Early detection and treatment of common variable immunodeficiency disorder (CVID) related granulomatous-lymphocytic interstitial lung disease (GLILD) is paramount in limiting long-term complications such as pulmonary fibrosis and mortality. While GLILD is among the most serious pulmonary complications of CVID, its risk factors have not been studied. The purpose of this study was to identify potential risk factors for GLILD, including clinical characteristics, peripheral B and T cell blood profiles, and pulmonary physiology.

METHODS: From the Interstitial Lung Disease (ILD) and National Jewish Health (NJH) research database, we identified seventeen consecutive adult CVID patients with radiologic-pathologic GLILD evaluated at NJH between 2000 and 2012. Each case was matched to one CVID control selected at random from the pool of all potential controls matched for age (within five years) and gender. We used logistic regression to determine independent predictors of GLILD.

RESULTS: The median time from CVID diagnosis to GLILD detection was eight years. Dyspnea on exertion was present in all GLILD patients. Compared with matched controls, cases were more likely to have a history of smoking, lymphadenopathy, autoimmune cytopenias, hypersplenism, polyarthritis, lower switched and memory B cells, and restrictive lung function before GLILD diagnosis. Multivariate analysis revealed that hypersplenism (OR = 28, CI 95% 1.8-480) and polyarthritis (OR = 23, CI 95% 1.3-177) were strongly associated with GLILD.

CONCLUSIONS: The presence of extrathoracic manifestations such as hypersplenism and polyarthritis are strong risk factors for GLILD in CVID patients and should alert clinicians to screen for lung involvement in these patients.

CLINICAL IMPLICATIONS: Patients with CVID and hypersplenism and/or polyarthritis have increased likelihood of developing interstitial lung disease specifically GLILD. The clinical implication is that these patients should be monitored and screened closer for developing interstitial pulmonary disease to aid in early diagnosis and treatment.

DISCLOSURE: The following authors have nothing to disclose: Richard Hedelius, Amar Mannina, Joshua Solomon, Jeffrey Swigris, Evans Fernandez

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