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Diffuse Lung Disease |

Granulomatous Lymphocytic Interstitial Lung Disease With Organizing Pneumonia in a Patient With Common Variable Immunodeficiency

Shraddha Goyal, MBBS; Robert Lenox, MD; Christopher Curtis, MD; Abbasi Adnan, MD
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SUNY Upstate Medical University, Syracuse, NY


Chest. 2015;148(4_MeetingAbstracts):368A. doi:10.1378/chest.2278711
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Abstract

SESSION TITLE: Diffuse Lung Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Common Variable Immunodeficiency (CVID) is the inherited primary immunodeficiency disease characterized primarily by generalized failure of antibody production. The most common pulmonary manifestation is recurrent respiratory tract infection and bronchiectasis. About one-third of the patients can have noninfectious pulmonary manifestations.

CASE PRESENTATION: A 20-year-old male presented to the clinic with sore throat and non-productive cough. He denied fever or night sweats. He had CVID since the age of 10 years and history of immune thrombocytopenic purpura. He was receiving monthly intravenous immunoglobulin infusions. CT of the chest showed innumerable pulmonary nodules in bilateral lungs distributed randomly. There was enlargement of supraclavicular, mediastinal and hilar lymph nodes. He was known to have pulmonary nodules which were initially noted on CT scan of chest in 2005. He underwent open lung biopsy in 2005 and 2012 both of which showed features of benign lymphoid hyperplasia. The recent CT chest showed increased number of nodules with prominent lymphadenopathy. Due to concern for lymphoma, he underwent left upper lobe VATS biopsy. The pathology showed lymphoid hyperplasia, poorly formed granulomas and fibroblastic foci. The immunological stain of lymphoid tissue expressed both T and B-cells suggesting benign etiology. The pathology was consistent with granulomatous-lymphocytic interstitial lung disease with co-existent organizing pneumonia. The tissue cultures were negative for bacterial, fungal and mycobacteria.

DISCUSSION: The granulomatous and lymphoproliferative histopathologic patterns such as lymphocytic interstitial pneumonia, follicular bronchiolitis, and lymphoid hyperplasia have been grouped under the term granulomatous-lymphocytic interstitial lung disease (GLILD). Organizing pneumonia has been rarely reported to occur along with GLILD. Radiologic findings in GLILD may include lung nodules, ground glass opacities, pulmonary consolidation, bronchiectasis, bronchial wall thickening and mediastinal lymphadenopathy. Presence of GLILD in patients with CVID is associated with worse prognosis. There are no controlled trials for effective treatment but corticosteroids have shown to benefit

CONCLUSIONS: Granulomatous-lymphocytic interstitial lung disease (GLILD) associated with CVID is relatively unstudied pathological entity.

Reference #1: Granulomatous-lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID) Joon H. Park, Section of Allergy and Immunology, October 2009

DISCLOSURE: The following authors have nothing to disclose: Shraddha Goyal, Robert Lenox, Christopher Curtis, Abbasi Adnan

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