A CASE OF COMMON VARIABLE IMMUNODEFICIENCY (CVID) COMPLICATED BY GRANULOMATOUS LYMPHOCYTIC INTERSTITIAL LUNG DISEASE (GLILD) TRIGGERED BY RECENT COVID-19 INFECTION

ABSTRACT

SESSION TITLE Unique Inflammatory and Autoimmune Complications of COVID-19 Infections SESSION TYPE Rapid Fire Case Reports PRESENTED ON 10/19/2022 1245 pm - 145 pm INTRODUCTION: Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder characterized by low serum IgG, IgA and/or IgM, and poor specific antibody production. CVID is estimated to affect as many as 1 in 25,000 individuals. Chronic lung disease is a common problem in patients with CVID. About 10-20% of patients have lymphocytic infiltrates and/or sarcoid-like granulomas, with several histological findings, termed granulomatous and lymphocytic interstitial lung disease (GLILD). CASE PRESENTATION Patient is a 61-year-old Caucasian woman with a history significant for CVID in remission who presented to the Pulmonary Clinic with a chief compliant of dyspnea of exertion (DOE). Patient was not suffering from any respiratory complaints until the diagnosis of severe COVID-19 pneumonia 4 months prior. For the following months, patient was slowly improving but was still suffering from severe DOE that has negatively impacted her quality of life. Patient has a remote history of smoking, having quit 10 years ago. Patient denied any joint pain, stiffness, swelling, skin rash, muscle ache, or weakness. No known history of SLE, Rheumatoid Arthritis, or other collagen vascular disorders had been reported. Patient denied any exposure to birds. Physical exam was significant only for bilateral basal rales with no wheezing or crackles. No skin rash, joints deformities, or clubbing was noticed. Laboratory studies revealed ESR was 17 with a CRP of 10.6. Negative ANA, SM, RNP, and SSA/SSB antibodies. Her Immunoglobulins levels were low with IgG 382 (nl > 610) and IgA < 2 (nl > 85). Her PFT revealed severe restrictive process with TLC 46% of predicted and severe reduction in DLCO at 35%. CT chest revealed diffuse central groundglass opacities, and interstitial thickening with traction bronchiectasis. Lung biopsy via VATS revealed lung parenchymal with focal, noncaseating granulomas, foci of focal interstitial lymphocytic infiltration and fibrosis;features consistent with Granulomatosis-Lymphocytic Interstitial Lung Disease (GLILD). Systemic steroid initiated and for the following weeks patient reports significant improvement in DOE. Her PFT at 3 month follow up showed significant improvement in FVC (5% increase), TLC (11% increase), and DLCO (5% increase). DISCUSSION: The respiratory manifestations of CVID follow two main mechanisms injury due to acute or recurrent infections and damage due to poorly understood immune-mediated processes. Severe COVID-19 results in dysregulated immune and inflammatory response that can worsen an underlying lung disease. Previous cases have been reported about CVID with GLILD complicated with COVID-19 infection but not vice versa. CONCLUSIONS: To our knowledge, this is a rare case of CVID complicated by GLILD triggered by recent COVID-19 infection. However, little is known about the association between COVID-19 infection and GLILD and further investigation is needed. Reference #1 Ho HE, Mathew S, Peluso MJ, Cunningham-Rundles C. Clinical outcomes and features of COVID-19 in patients with primary immunodeficiencies in New York City. J Allergy Clin Immunol Pract. 2020;S2213–2198(20)31102–8. Reference #2 Prasse A, Kayser G, Warnatz K. Common variable immunodeficiency-associated granulomatous and interstitial lung disease. Curr Opin Pulm Med. 2013;19503–9. Reference #3 Cunningham-Rundles C, Bodian C. Common variable immunodeficiency clinical and immunological features of 248 patients. Clin Immunol. 1999;9234–48. DISCLOSURES No relevant relationships by husam nayef No relevant relationships by Arshia Vahabzadeh No relevant relationships by Zaid Yaqoob No relevant relationships by Mohammad Zalt