key: cord-0044631-g7j4po0n authors: Aljaberi, Rana; Wishah, Kholoud title: Positive Outcome in a COVID-19 patient with Common Variable Immunodeficiency after IVIG date: 2020-06-04 journal: Ann Allergy Asthma Immunol DOI: 10.1016/j.anai.2020.06.006 sha: e13bddfacb1ff5bfc15e939cf95432f278b4cfdd doc_id: 44631 cord_uid: g7j4po0n nan We present a case of an immunodeficient patient patient who has recovered from COVID-19 1 infection. This is a 53-year-old female with Common Variable Immunodeficiency diagnosed in 2 1996 and has been on regular IVIG replacement therapy, GAMUNEX®-C 40 grams (520 mg/ 3 kg/ dose) every 2 weeks. Last IgG trough level was 1478 mg/dl on 11-12-19. High dose 4 influenza vaccine administered in September 2019. The patient has also hypothyroidism, 5 bronchiectasis, and Sjogren's Syndrome treated with hydroxychloroquine 400 mg daily. Last On day 2 of hospitalization, she required 2 liters/minute oxygen by nasal cannula. On day 5, 20 patient had increased oxygen requirement and was transferred to the Intensive Care Unit. Her 21 respiratory status worsened and needed escalation of support to NIPPV/CPAP and ultimately 22 intubation and mechanical ventilation on hospital day 7. Her medical treatment included 23 ceftriaxone and doxycycline for the duration of hospitalization as well as hydroxychloroquine, 24 which was increased from her home regimen to 600 mg/day. She was successfully weaned and 25 extubated on hospital day 13. On day 14, a second dose of 40 g IVIG was administered, after 26 which, the patient was discharged home to self-quarantine due to repeat positive COVID-19 27 testing. The patient never received any convalescent COVID 19 plasma. 28 The underlying pathophysiology of COVID-19 is under investigation in animal models. It 30 appears that the virus induces an inflammatory response involving macrophage hyperactivation, 31 leading to a cytokine storm responsible for severe lung and systemic complications, making 32 IVIG anti-inflammatory effect potentially useful in treating COVID-19 1,2 , especially in cases of 33 severe COVID-19 cases associated with lymphopenia and increased cytokine levels 3 . 34 We present a case of COVID-19 at very high risk for morbidity and mortality secondary to 36 underlying immunodeficiency and bronchiectasis. Despite presenting with classical pneumonia 37 requiring intubation and mechanical ventilation, the patient recovered completely and had a 38 relatively short hospital course. Patient who have had similar courses have had a reported 39 mortality rate of 49-97% 4,5 . It is hard to ascertain if this was a result of the hydroxychloroquine, 40 high dose IVIG, or a combination of both. IVIG has been shown to have an immunomodulatory, anti-41 inflammatory effect especially if given in higher doses, the exact mechanism is still unknown, but it has 42 been suggested that it occurs through Fc-mediated mechanism, or Fab-mediated mechanisms 6. Azithromycin was not included in her therapy, due to a history of allergic reaction to the 44 antibiotic. Hydroxychloroquine has been shown to be associated with viral load reduction/ 45 disappearance and its effects reinforced by azithromycin in a small group of patients 7 However, 46 its efficacy in improving clinical course has yet to be determined. Our findings suggest that early administration of IVIG may be beneficial in improving the 48 outcome of this illness, especially in patients with an immunodeficiency disorder. A similar 49 report of three patients from China showed that high dose IVIG had a significant impact on 50 improving symptoms, fever curve, and lymphopenia 8 , although the selected patients in that 51 report were not immune deficient. We speculate that IVIG, in addition to its immunomodulatory 52 effect, may contain antibodies to other coronaviruses that are cross reactive with COVID-19. 53 This might lead to modulation of the severity of the illness similar to what is observed in 54 pediatric population who, in general, present with milder form of this disease that is speculated 55 to be secondary to prior exposure to other coronavirus infection. COVID-19: the new challenge for rheumatologists COVID-19: consider cytokine storm syndromes and immunosuppression SARS-CoV-2: A Storm is Raging Clinical course and risk factors for mortality of adult inpatients with COVID-19 in Wuhan, China: a retrospective cohort study Characteristics of and important lessons from the coronavirus disease 2019 (COVID-19) outbreak in China: summary of a report of 72 314 cases from the Chinese Center for Disease Control and Prevention Immunomodulation by IVIg and the role of Fc-gamma receptors: classic mechanisms of action after all? Hydroxychloroquine and azithromycin as a treatment of COVID-19: results of an open-label non-randomized clinical trial High-dose intravenous immunoglobulin as a therapeutic option for deteriorating patients with Coronavirus Disease