Abstract Common variable immunodeficiency (CVID) describes a heterogenous group of disorders of hypogammaglobulinemia of unknown etiology. It is the most common primary immunodeficiency in the United States. It can present in childhood, but is often diagnosed in adulthood. Upon presentation, patients are usually found to have recurrent severe bacterial infections or prolonged infections that do not respond to antibiotics. Etiology of CVID is unclear but genetic inheritance is found in about 20-30% of patients. Diagnosis is made when three criteria are met: a marked decrease of IgG and of at least one of the IgM or IgA subtypes, previously poor response to vaccinations, and other defined causes of hypogammaglobulinemia have been eliminated. This case presents a 59-year-old female with a history of asthma and recurrent parapneumonic effusions who presented to the ED after three days of epigastric pain and one day of shortness of breath. Initial vital signs showed a heart rate of 118 beats/min, a respiratory rate of 30 breaths/min, normotensive blood pressure, and oxygen saturation of 100% on 2 L Nasal Cannula. Laboratory evaluation showed a WBC of 22.1× 103/uL, hemoglobin of 11.1 g/dL, elevated inflammatory markers, creatinine of 2.1 mg/dl, and arterial lactate of 2.3 mmol/L. EKG showed diffuse ST elevation. CTA chest showed pericardial effusion and right lower lung pneumonia with partially loculated right sided pleural effusion. Bedside TTE confirmed pericardial effusion with no signs of tamponade physiology. Blood cultures were drawn, and the patient was started on broad spectrum antibiotics. Pulmonology was consulted for the loculated pleural effusion and cardiology for the pericardial effusion. She was found to have culture negative purulent pericarditis via pericardiocentesis and a streptococcus pneumoniae empyema via thoracentesis. Due to the severity of her presentation, there was concern for immunodeficiency, so workup was initiated. Laboratory workup was notable for all her immunoglobulin levels being below assay. Additionally, the patient had no antibody response to Strep pneumoniae despite receiving vaccination 1 year prior. The patient had a prolonged hospital course, but with an interdisciplinary workup with infectious disease, pulmonology, and cardiology resulted in presumed diagnosis of combined variable immunodeficiency (CVID). Following discharge, she had close follow up with Allergy/Immunology and was started on Intravenous Immunoglobulin. In summary, this case serves as an example of the low threshold that should be used when considering a primary immunodeficiency disorder such as CVID as the prevalence is likely much greater than commonly thought. This abstract is funded by: none
Crossland et al. (Fri,) studied this question.