SESSION TITLE: Infectious Disease Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Our case demonstrates a rare immunodeficiency as the cause of Aspergilloma and describes the medical and surgical management.
CASE PRESENTATION: A 29 year old woman with a history of hyper-IgE syndrome with recurrent skin and lung infections presents with shortness of breath and wheezing. Three months prior, she was diagnosed with a pneumonia based upon right upper lobe consolidation on chest x-ray and treated with multiple antibiotics. Despite management, her cough with tenacious secretions and shortness of breath persisted. Physical exam showed inspiratory wheezing and restricted chest wall motion. A 4 by 7 centimeter septated, thick-walled cavity in the superior segment of the right lower lobe with dependent debris suggestive of an Aspergilloma was seen on Chest CT (image 1). Immunoglobin E level was 11,667, positive Galactomannan assay, and peripheral eosinophilia was 16% (1.17 K/uL). She was treated with Posaconazole and referred to thoracic surgery. A right lower lobectomy and partial resection of the posterior segment of the right upper lobe was performed (image 2). The patient had an uncomplicated recovery, reduced symptoms, and returned to work and exercise. Pathology was consistent with an Aspergilloma with cultures growing A. fumigatus.
DISCUSSION: Hyper-IgE Syndrome, known as Job’s Syndrome is associated with a dominant STAT3 mutation, a regulator of the immune response.1 Hyper-IgE syndrome subjects develop pneumatoceles, likely due to aggressive and dysregulated inflammation.1 The mortality rate of patients with fungal disease is estimated at 16.7% and in a review, four of six deaths were due to A. fumigatus lung infections.2,3 Surgical complications include air leaks and poor lung expansion.1 Our patient was referred for surgical resection based upon the large Aspergilloma size and the likelihood of worsening with conservative therapy. Post-procedure, the patient was continued on Posaconazole; while there is little data to support prophylactic anti-fungals our patient may have benefited from its use.1
CONCLUSIONS: Invasive fungal infections are a major cause of mortality in Job's Syndrome.
Reference #1: Freeman AF, Holland SM. The hyper-IgE syndromes. Immunology and allergy clinics of North America 2008;28:277-91, viii.
Reference #2: Vinh DC, Sugui JA, Hsu AP, Freeman AF, Holland SM. Invasive fungal disease in autosomal-dominant hyper-IgE syndrome. J Allergy Clin Immunol 2010;125:1389-90.
Reference #3: Freeman AF, Kleiner DE, Nadiminti H, et al. Causes of death in hyper-IgE syndrome. J Allergy Clin Immunol 2007;119:1234-40.
DISCLOSURE: The following authors have nothing to disclose: Benjamin Wu, Gail Schattner
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