Allergy and Airway: Student/Resident Case Report Poster - Allergy and Airway |

ANCA-Negative Eosinophilic Granulomatosis With Polyangiitis FREE TO VIEW

Rizwan Khan, MD; Vickram Tejwani, MD; Sumita Khatri, MD
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Cleveland Clinic Foundation, Cleveland, OH

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

Chest. 2016;150(4_S):17A. doi:10.1016/j.chest.2016.08.020
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SESSION TITLE: Student/Resident Case Report Poster - Allergy and Airway

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA), alternatively named Churg Strauss syndrome (CSS), is a systemic disorder characterized by asthma, transient pulmonary infiltrates, hypereosinophilia and systemic small and medium vessel vasculitis.

CASE PRESENTATION: A 63 year old female presented for an evaluation of sinus congestion and pressure along with rhinorrhea for the past 18 months. Symptoms gradually progressed to dyspnea and a productive cough necessitating multiple hospitalizations. Symptoms were elicited by cold air and exercise. Prior therapies included inhaled albuterol, budesonide and ipratropium, as well as anti-histamines and oral steroids. She also had significant neuralgias along with blurry vision. Blood work showed eosinophilia (16.8%) with increased IgE (352). c-ANCA and p-ANCA were persistently negative. Intradermal test was negative for Aspergillus. Sinus CT revealed opacification of sinuses bilaterally for which she underwent septoplasty and functional endoscopic sinus surgery. Tissue pathology revealed chronic inflammation and eosinophils. CT chest revealed patchy ground glass opacities which were transient. PFTs showed an FEV1/FVC ratio of 67%, FEV1 83% predicted, with a 19% bronchodilator reversibility. Bronchoscopy with BAL was positive for Aspergillus, but negative for eosinophils.

DISCUSSION: EGPA is a granulomatous inflammation of small -medium vessels characterized by three successive stages initially manifesting as asthma which progresses to the hypereosinophilic stage before developing into systemic vasculitis. Severity and prognosis are determined by the FFS which includes age >65, GI involvement, cardiac insufficiency, renal insufficiency and absence of ENT symptoms. ANCA antibodies are positive in around 40% of patients and are associated with renal involvement as well as other systemic manifestations including pulmonary involvement.1 On the other hand, ANCA negative patients have greater cardiac manifestations of EGPA.1 They are also more likely to suffer from much severe asthma prior to EGPA diagnosis.2 Over 90% of patients with an FFS of zero such as our patient achieve remission with corticosteroid therapy alone.3

CONCLUSIONS: Early diagnosis and treatment of EGPA are essential to reduce morbidity from severe systemic manifestations.

Reference #1: Sokolowska BM, et al. ANCA-positive and ANCA-negative phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA): outcome and long-term follow-up of 50 patients from a single Polish center. Clin Exp Rheumatol. 2014 May-Jun;32(3 Suppl 82):S41-7.

Reference #2: Sinico RA, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis and Rheumatism. 2005;52(9):2926-2935.

Reference #3: Ribi C, Cohen P, Pagnoux C, et al. French Vasculitis Study Group. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum. 2008;58:586-586.

DISCLOSURE: The following authors have nothing to disclose: Rizwan Khan, Vickram Tejwani, Sumita Khatri

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