Granulomatous Lymphocyte Interstitial Lung Disease (GLILD): A Rare Complication of Common Variable Immunodeficiency (CVID)

Document Type

Conference Proceeding

Publication Date

5-2024

Publication Title

American Journal of Respiratory and Critical Care Medicine

Abstract

Introduction Granulomatous lymphocytic interstitial lung disease (GL-ILD) is a rare, non-infectious extra-pulmonary manifestation of common variable immunodeficiency (CVID). Diagnosing and managing GLILD remains challenging due to poorly understood pathogenesis and high mortality. We present a complex case of a young female with CVID associated with lung and spinal cord involvement. Case presentation A 39-year-old female with a medical history of ITP, and CVID on monthly intravenous immunoglobulins infusions presenting with one year history of right leg numbness. Spine MRI showed a hyperintense T2 signal within the upper thoracic spinal cord without enhancement or active lesions. Chest x-ray showed bilateral hilar lymphadenopathy. She was initially diagnosed with neurosarcoidosis. A high-resolution chest CT scan showed mid and lower lobe predominant peribronchovascular groundglass and consolidative nodular opacities (figure). The patient was asymptomatic from a pulmonary standpoint and not hypoxic. She underwent VATS wedge lung biopsy, and pathology showed lymphoid aggregates and secondary germinal centers resulting in mass-forming lesions and collagen deposition around terminal airways diagnostic of GLILD. Further workup was unremarkable, including bronchoscopy with BAL and pulmonary function testing. Lumbar puncture showed no oligoclonal bands and no evidence of multiple sclerosis. The etiology of her neurological symptoms was more in favor of granulomatous myelitis seen in CVID. She was started on pulse followed by taper steroid therapy. Multidisciplinary team decided to proceed with rituximab 375mg/m2 weekly for four weeks, then every six months for two years, and azathioprine daily. Plan for close follow-up for clinical and radiological improvement on future high-resolution CT scans. Discussion GL-ILD is a subset of lymphocytic interstitial pneumonia leading to interstitial lung disease; it occurs in up to 10-20% of patients with CVID. GL-ILD can be frequently misdiagnosed as sarcoidosis. However, the presence of radiological lower lobe distribution can help in differentiating it. VATS is the gold standard method to diagnose GL-ILD, as it allows for sufficient tissue evaluation and excludes other conditions like lymphoma. Besides glucocorticoids, there has yet to be a consensus on the optimal treatment for GL-ILD. Neurological involvement in our patient favored a steroid-sparing immunosuppressant agent with neural tissue penetration. The presence of GL-ILD shortens survival in CIVD patients. Without treatment, GL-ILD can lead to irreversible pulmonary fibrosis.

Volume

209

Issue

Suppl

First Page

A5858

Comments

International Conference of the American Thoracic Society, May 17-22, 2024, San Diego, CA

DOI

10.1164/ajrccm-conference.2024.209.1_MeetingAbstracts.A5858

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