SESSION TITLE: Chest Infections Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: There is often a critical delay in the diagnosis of primary immunodeficiency syndromes which contributes to their morbidity and mortality. Good syndrome is the rare association between adult onset immunodeficiency and a thymoma.
CASE PRESENTATION: A Hispanic septuagenarian with a history of Candida esophagitis presented with a six month history of cough and weight loss despite courses of empiric antibiotics. Chest CT showed an anterior mediastinal mass and diffuse tree-in-bud opacities. The mediastinal mass was resected and pathology showed a benign thymoma. Bronchoscopy revealed purulent secretions with cultures growing Pseudomonas aeruginosa and Mycobacterium avium complex (MAC). Despite appropriate antibiotic therapy, he presented with repeated episodes of cough and weight loss. Chest CT revealed waxing and waning of pulmonary infiltrates for which he underwent repeated bronchoscopy. Bronchial wash cultures grew Moraxella catarrhalis, Nocardia asteroides complex, Staphylococcus aureus, Escherichia coli, Cladosporium species, and Candida albicans on different occasions despite tailored antibiotic therapy. Given these recurrent infections, immunologic studies were obtained. They revealed a low CD4 count though HIV negative and undetectably low IgG4 levels with normal total IgG, IgM, IgA, and IgE levels. Given his history of a thymoma and evidence of cellular and humoral deficiency, a diagnosis of Good syndrome was made and the patient was started on monthly intravenous immunoglobulin therapy in addition to antibiotic therapy.
DISCUSSION: Good syndrome is characterized by a thymoma, recurrent sinopulmonary or opportunistic infections, decreased serum immunoglobulins, and reduced or absent B cells. Clinical presentation is varied but most commonly, patients suffer from recurrent sinopulmonary infections, especially due to encapsulated organisms, and opportunistic viral or fungal infections, particularly cytomegalovirus and mucocutanous Candida infection. Management of Good syndrome includes thymoma resection, aggressive antibiotic therapy, and immunoglobulin replacement therapy.
CONCLUSIONS: Good syndrome is the combination of a thymoma and adult onset immunodeficiency. Immunodeficiency syndromes are an under-recognized etiology of recurrent pulmonary infections. Early recognition is crucial to limiting their morbidity and mortality.
Reference #1: Agarwal S et al. Thymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of the literature. Ann Allergy Asthma Immunol 2007;98(2):185-190.
DISCLOSURE: The following authors have nothing to disclose: Sheila Habib, Tamara Simpson
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