A NEAR MISS IS AS GOOD AS A MILE: RECOGNIZING HYPEREOSINOPHILIC SYNDROME

Abstract

<h3>Introduction</h3> We discuss a case of hypereosinophilic syndrome (HES) misdiagnosed as acute chest syndrome (ACS). Learning objectives include recognizing premature closure as a cognitive bias and review of diagnostic criteria for Eosinophilic Granulomatosis with Polyangiitis (EGPA). <h3>Case Description</h3> A 46-year-old woman with asthma and sickle-cell disease (SCD) complained of worsening nasal congestion, productive cough, and shortness of breath persisting despite multiple courses of antibiotics. She underwent sinus surgery, and microscopy of ethmoid sinus contents revealed eosinophilic inflammation. She developed worsening cough and shortness of breath and was treated with antibiotics for presumed pneumonia. Her respiratory status deteriorated, requiring intubation. CXR revealed patchy opacification concerning for ACS. AEC was 4320 cells/µL. She was started on methylprednisolone and successfully extubated. The diagnosis of EGPA was established, and she was transitioned to mepolizumab. Symptoms have improved on this treatment. <h3>Discussion</h3> ACS is diagnosed in a patient with SCD when a new pulmonary infiltrate is present on chest imaging. This is frequently attributed to complications of a vaso-occlusive crisis or infection, as it was initially in this case. However, given her history of asthma, peripheral eosinophilia and recent pansinusitis with eosinophilic infiltration, a diagnosis of EGPA should be considered. In diagnostic reasoning, physicians aim to quickly develop a working diagnosis based on history and objective data in attempt to treat the presenting condition. However, one must be careful to avoid premature closure, a type of cognitive error, which occurs when physicians accept an initial diagnosis and fail to contemplate other alternatives, leading to misdiagnosis and ultimate delay in treatment.