Seeing the Forest for the Trees: A Case of an Atypical Bacterial Pneumonia Leading to the Diagnosis of CVID

Abstract

SESSION TITLE: Other Infections SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Combined Variable Immunodeficiency Disorder (CVID) in adults is a rare but frequently lethal disease due to its low clinical suspicion and heterogeneous presentation. We present a patient in septic shock due to multifocal bacterial pneumonia who was ultimately diagnosed with CVID. CASE PRESENTATION: A 34-year old female with no significant past medical history presented for 2 days of worsening shortness of breath preceded by 2 weeks of productive coughs, fevers, chills, and fatigue. On presentation, she was found to have absent left lung sounds and underwent an immediate chest tube thoracostomy for a pneumothorax. A subsequent CT showing bilateral multifocal cystic consolidations and a large loculated effusion in the left lung base. Initial labs showed a moderate leukocytosis with neutrophilia. The patient was admitted to the ICU for septic shock secondary to a bilateral necrotizing pneumonia and required a second chest tube to drain her left lung effusion. Pleural fluid, blood, and sputum cultures grew out Prevotella and Achromobacter. The patient ultimately required emergent intubation for hypoxic respiratory failure, and was found to have severe hypogammaglobinemia correlating with CVID on labratory results. On later questioning, the patient recalled that she had recurrent annual sinus infections since adolescence and was once told by a physician she had an illness that’s description correlated with autoimmune hemolytic anemia. Allergy & Immunology and Infectious Disease were immediately consulted, and the patient was started on amphotericin B, ciprofloxacin, and meropenem in addition to undergoing 2 rounds of IVIG treatment. The patient was ultimately transferred out of the ICU in stable condition and discharged home with close follow-up for further workup of her CVID. DISCUSSION: The mean age of onset for CVID in females is 27-years old, with a typical delay in diagnosis of 6-7 years. The spectrum of initial presentation is wide, including sinopulmonary infections, autoimmune diseases, and even malignancy, making diagnosis difficult. However, early detection is essential as the mean age of mortality in females is 44-years old, with delay to diagnosis being one of the biggest risk factors for mortality. CONCLUSIONS: We present a case of a previously undiagnosed female with CVID presenting with severe bilateral necrotizing bacterial pneumonia requiring an extensive ICU stay with multiple invasive interventions. Early detection of her CVID may have prevented this near-fatal encounter, and serves as valuable evidence for the need to regularly consider immunodeficiency disorders in adults with recurrent infections. Reference #1: Gathmann B, Mahlaoui N, Ceredih, et al. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014;134(1):116-126. Reference #2: Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 2012;119(7):1650-1657. DISCLOSURE: The following authors have nothing to disclose: Lakshay Jain, Sami Bashour, Harman Kular, Simon Yau, Purvesh Patel, Nicola Hanania No Product/Research Disclosure Information