Critical Care: Critical Care |

The Job Syndrome Doing a Lethal Job FREE TO VIEW

Hammad Arshad, MD; Rajashekar Adurty, MD; Meilin Young, MD
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Allegheny General Hospital, Pittsburgh, PA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A136. doi:10.1016/j.chest.2016.02.142
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SESSION TITLE: Critical Care

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Hyper IgE syndrome is a primary immunodeficiency disorder characterized by recurrent infection of the skin and the lung (1).

CASE PRESENTATION: A 30-year-old African American male with past medical history of Job syndrome, recurrent pulmonary infections, pneumatoceles and cutaneous abscesses presented to our hospital with severe septic shock, DIC and multi-organ failure. A CT of the neck and chest showing small cutaneous abscess in the neck and cavitory lesion in the lung with multi-lobar involvement. The patient was intubated for Acute Respiratory Failure and started on vasopressor support and broad spectrum antibiotics. Further interventions and workup included drainage of the neck abscess and Echocardiogram that was negative for vegetations. Unfortunately, patient was septic with beta hemolytic streptococci bacteremia that didn’t respond to appropriate antibiotics. He eventually died of multiorgan failure

DISCUSSION: Hyperimmunoglobin E disease which was originally called Job syndrome characterized by the classic triad of eosinophilia, eczema and recurrent skin and pulmonary infections. It is an autosomal dominant disorder with a negative mutation in STAT 3 signalling molecule resulting in immune dys-regulation and increased IgE production. A scoring system that weighs both immunological and somatic features of the syndrome aid in establishing the diagnosis and predicting the mutation. The skin and pulmonary infection are generally moderate and respond to antimicrobial treatment with pneumatocele formation. Rarely do they prove lethal with isolated case reports of superinfection with pseudomonas and Aspergillus that were life threatening. (2)

CONCLUSIONS: Job disease is a rare disorder characterized by multi system and recurrent microbiological involvement. Aggressive antibiotics would need to be initiated early along with surgical drainage of abscesses for improved outcomes.

Reference #1: Grimbacher B et al; Hyper IgE syndrome with recurrent infections-an autosomal dominant multi system disorder; NEJM 1999;340

Reference #2: Freeman AF et al; Causes of death in hyper IgE Syndrome; J Allergy Clin Immunol 2007;119

DISCLOSURE: The following authors have nothing to disclose: Hammad Arshad, Rajashekar Adurty, Meilin Young

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