GRANULOMATOUS-LYMPHOCYTIC INTERSTITIAL LUNG DISEASE AS THE NONINFECTIOUS COMPLICATION OF COMMON VARIABLE IMMUNODEFICIENCY

ABSTRACT

SESSION TITLE Difficult Diffuse Lung Disease SESSION TYPE Rapid Fire Case Reports PRESENTED ON 10/18/2022 1015 am - 1110 am INTRODUCTION: Common Variable Immunodeficiency (CVID) is one of the most common humoral immunodeficiency disorders and usually manifests as infectious complications. However, noninfectious complications such Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) can convey a much poorer prognosis in patients with CVID. In this case report, we present a patient with GLILD who presented with cough and shortness of breath. CASE PRESENTATION A 66 year old female with past medical history of provoked DVT (off anticoagulation), cervical cancer (s/p resection) presented to pulmonology clinic with complaints of chronic cough and shortness of breath on exertion. She had a negative smoking history and no occupational exposures. She was up to date on vaccinations and COVID was negative. Vitals were stable and physical exam was normal including clear breath sounds. CXR revealed emphysema and hazy opacities in the lung bases. PFTs demonstrated moderate obstructive pattern with no response to bronchodilator, normal lung volumes, and DLCO of 76%. Due to lack of improvement in her cough, CT Chest was done which revealed diffuse pulmonary nodules, bronchiectasis with possible atelectasis or scarring in the RML and lingula, and a prominent subcarinal lymph node. EBUS TBNA of station 7 returned negative for malignancy. Culture showed polymicrobial growth with negative AFB and fungi. Patient was treated without antibiotics, but due to family history of immunodeficiency, immunoglobulin panel was sent which returned low IgG subclasses. She then received IVIG. However, given the centrilobular nodules and lack of response to IVIG, repeat bronchoscopy with TBBx and BAL was performed. BAL revealed lymphocytic predominance and tissue biopsy showed non-caseating granulomas and negative cultures. Eventually patient was diagnosed with GLILD and started on 6 weeks of prednisone 40 mg daily along with PJP prophylaxis. However, her symptoms remained same and rituximab was prescribed with improvement in the symptoms. DISCUSSION: Although recurrent sinopulmonary infections are common in CVID patients, if clinical response to IVIG is minimal to none, GLILD should be considered on the differential. Centrilobular nodules and ground glass opacities should raise suspicion of GLILD and tissue sample should be obtained in these patients to confirm the diagnosis. Appropriate treatment with prednisone or rituximab along with IVIG improves GLILD patient symptoms and yields better outcomes in terms of morbidity and quality of life. CONCLUSIONS: Appropriate treatment with prednisone or rituximab along with IVIG improves GLILD patient symptoms and yields better outcomes in terms of morbidity and quality of life. Reference #1 Hurst JR, Verma N, Lowe D, Baxendale HE, Jolles S, Kelleher P, et al. British lung foundation/United Kingdom primary immunodeficiency network consensus statement on the definition, diagnosis, and management of granulomatous-lymphocytic interstitial lung disease in common variable immunodeficiency disorders. J Allergy Clin Immunol Pract. (2017) 5938– 45. doi 10.1016/j.jaip.2017.01.021 DISCLOSURES No relevant relationships by Benjamin Butler No relevant relationships by Abdulmetin Dursun No relevant relationships by Badri Giri No relevant relationships by Emily Smallwood