Via Research Recognition Day 2024 VCOM-Carolinas

Clinical Case-Based Reports

Epiglottitis Strikes Twice: An Adult Case of Recurrent Epiglottitis Brooke Escoe, DO, PGY-1 1 , Brody M. Fogleman, OMS-III 2 , Robert Sherertz, MD 1

1 Grand Strand Medical Center, Department of Internal Medicine, Myrtle Beach, SC 2 Edward Via College of Osteopathic Medicine – Carolinas, Spartanburg, SC

Case Summary

Abstract

Discussion

Context: The incidence of epiglottitis in children has drastically decreased due to the H. influenzae type b vaccine 1 , while in adults, it remains relatively constant. Recurrent adult epiglottitis is rare, possibly linked to impaired humoral or cell mediated immunity. Case Presentation: A 58-year-old male with type 2 diabetes mellitus had a second episode of acute epiglottitis 9 years after the first. Treatment with vancomycin, ceftriaxone, and dexamethasone yielded an excellent response. The patient exhibited reduced serum IgG and a low CD4 lymphocyte count, and multiple negative HIV tests. Comments: Literature review identified risk factors for recurrent epiglottitis in adults, excluding idiopathic CD4 lymphopenia. This case suggests CD4 lymphopenia as a potential risk factor for recurrent epiglottitis in adults. Diagnosis: The patient's recurrent epiglottitis is likely due to a combined T-cell, B-cell immunodeficiency disorder. Preliminary findings hint at atypical HIV or idiopathic CD4 lymphopenia. This is the first report linking CD4 lymphopenia to recurrent epiglottitis in adults. v Pediatric Incidence: Primarily associated with Haemophilus influenzae type b (Hib), epiglottitis has seen a dramatic decline— 200-fold reduction from 4.9 to 0.02 cases per 100,000—attributed to the Hib vaccine. 2,3 v Adult Incidence: Relatively stable at around 2 to 4 cases per 100,000 3 , with recurrent episodes being exceptionally rare at approximately 1 case per 1,000,000. 4 Background

First report of CD4 lymphopenia as a potential risk factor for recurrent epiglottitis in adults.

Case Presentation: § 58-year-old male with insulin-dependent T2DM with progressively worsening fever, deep throat pain, and malaise for three days. § Previous episode of epiglottitis 9 years prior. § Initial vitals: HR 134/min, T 102.8 F, O 2 sat 99% on RA. § Leukocytosis (12.8 K/mm 3 ). § Negative blood cultures. § Negative for influenza A/B, SARS-CoV-2, and group A streptococcus. § Multiple negative HIV tests in the preceding 9 years. § Otolaryngology was consulted and acute recurrent epiglottitis was confirmed via CT imaging and flexible nasopharyngoscopy. § 3-day hospitalization with favorable response to ceftriaxone, vancomycin, and dexamethasone without intubation required.

#1

References Management Recommendations: § If the patient were willing, we would recommend the following: § HIV viral load § Baseline antibody level assessments, vaccination with conjugated Hib and pneumococcal vaccines, and follow-up antibody level assessments § CD4 lymphocyte counts and quantitative IgG levels every six months. § Follow-up with an infectious disease physician Significance and Limitations: § Expands understanding of recurrent adult epiglottitis and emphasizes CD4 lymphopenia as a possible risk factor. § Inability to definitively identify causative agents due to the absence of throat cultures, despite earlier presumptions of bacterial and fungal involvement. § Patient was unwilling to undergo HIV viral load and IgG subclass testing. Conclusion: § While recurrent adult epiglottitis is commonly linked to factors like alcohol use and diabetes, this case prompts new inquiries about the role of immunodeficiency disorders, specifically CD4 lymphopenia, in recurrent episodes. Possible underlying etiologies include long-term non-progressor elite controllers with HIV infection or ICL. 1. Hanna J, Brauer PR, Berson E, Mehra S. Adult epiglottitis: Trends and predictors of mortality in over 30 thousand cases from 2007 to 2014. Laryngoscope . 2019;129(5):1107-1112. 2. Hanna J, Brauer PR, Berson E, Mehra S. Adult epiglottitis: Trends and predictors of mortality in over 30 thousand cases from 2007 to 2014. Laryngoscope. 2019;129(5):1107-1112. 3. Guldfred LA, Lyhne D, Becker BC. Acute epiglottitis: epidemiology, clinical presentation, management and outcome. J Laryngol Otol. 2008;122(8):818-823. 4. Gu X, Gao F, Wang X. [Clinical characteristics and risk factors of recurrent acute infectious epiglottitis in adults]. Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2023;37(1):47-51. 5. Felton P, Lutfy-Clayton L, Smith LG, Visintainer P, Rathlev NK. A Retrospective Cohort Study of Acute Epiglottitis in Adults. West J Emerg Med. 2021 Nov 5;22(6):1326-1334. doi: 10.5811/westjem.2021.8.52657. PMID: 34787558; PMCID: PMC8597686. 6. Tsai YT, Huang EI, Chang GH, et al. Risk of acute epiglottitis in patients with preexisting diabetes mellitus: A population-based case-control study. PLoS One . 2018;13(6):e0199036. 7. Dowdy RAE, Cornelius BW. Medical Management of Epiglottitis. Anesth Prog. 2020 Jun 1;67(2):90-97. doi: 10.2344/anpr-66-04-08. PMID: 32633776; PMCID: PMC7342809. The patient provided written informed consent during his hospitalization for the utilization of his medical history and clinical findings for research and educational purposes. Patient specific data were obtained from electronic medical records of Grand Strand Medical Center. The authors declare that they have no financial or competing conflicts of interest regarding this research. Consent and Conflicts of Interest Very likely secondary to CD4 lymphopenia and/or hypogammaglobulinemia Uncomplicated acute recurrent epiglottitis Further research warranted to solidify associations

Figure 1. CT neck with contrast revealed asymmetric thickening of the right aryepiglottic fold. Confirmed via direct visualization.

Immunologic Insights: § Decreased serum IgG level suggest impaired humoral immunity. § Markedly low CD4 count suggest impaired cell-mediated immunity. § Multiple negative HIV tests, at-least one test yearly for nine years, and a negative test at follow-up = high cumulative negative predictive value.

CD4 lymphopenia: A condition characterized by an abnormally low count of CD4 T lymphocytes, indicative of compromised immune function, commonly associated with HIV but also observed in other immunodeficiency disorders. Idiopathic CD4 lymphocytopenia (ICL): A rare immunodeficiency disorder characterized by persistently low levels of CD4 T lymphocytes without HIV infection or another immunodeficiency disorder. Diagnosis of exclusion. CD4

v Risk factors for acute epiglottitis in adults: 4,5,6 § Diabetes mellitus § COPD § Tonsillar infection § Sjogren’s syndrome § Frequent alcohol use v Management strategies: 7 § Airway monitoring, with intubation and mechanical ventilation if necessary § Corticosteroids § Combination antibiotics

Patient's Immunologic Profile

IgG

IgA

28.9 20 25 30 35 40 45 50 55 60 CD4 Lymphocyte Proportion (%) % CD4

Absolute CD4 Count

IgM

1000 1100 1200 1300 1400 1500 1600 1700

100 150 200 250 300 350 400 450 500

120 150 180 210 240 270 300

800 1000 1200 1400

0 200 400 600

175.8

0 30 60 90

600 700 800 900

61

0 50

77

635 Measured Immunoglobulin Level (mg/dL)

This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities.

Absolute CD4 Lymphocyte Count (cells/mcL)

Figure 2. Depicts the patient's immunologic profile at the time of the second presentation. Gray shaded regions within each panel illustrate the expected range for the respective laboratory parameter.

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2024 Research Recognition Day