SHM Converge Scientific Abstract ePoster Gallery-A Case of Mpo-Anca Positive Granulomatosis With Polyangiitis Presenting With Subglottic Stenosis

Pdf Summary

This case describes a 23-year-old man who presented with sudden dyspnea, throat tightness, cough, and dysphonia during exercise. His symptoms were atypical for asthma and did not improve with albuterol. Although he had recent upper respiratory symptoms and partial improvement with racemic epinephrine and dexamethasone, outpatient ENT laryngoscopy later revealed subglottic stenosis, prompting re-admission for concern for croup or another upper-airway process.<br /><br />His history included exercise- and allergy-induced asthma, sinusitis, intermittent hematuria/proteinuria since childhood, dysphagia, nephrolithiasis, and GERD. Exam showed dysphonia but no stridor, and labs plus chest X-ray were unremarkable. CT neck demonstrated circumferential thickening and narrowing of the proximal subglottic airway. Repeat laryngoscopy showed nasal crusting, a sharp epiglottis, and subglottic edema. Infectious workup was negative.<br /><br />Because of persistent airway symptoms and imaging findings, the differential broadened to include autoimmune disease, particularly granulomatosis with polyangiitis (GPA). He was treated inpatient with ampicillin-sulbactam, dexamethasone, fluticasone, racemic epinephrine, and azelastine. He improved by day 3 and was discharged on TMP-SMX and prednisone. Later serologies showed ANA 1:320, p-ANCA 1:80, and elevated anti-MPO antibody, with negative c-ANCA and anti-PR3. Renal biopsy showed no significant kidney involvement. He ultimately required tracheal dilation for persistent subglottic stenosis.<br /><br />This case highlights that GPA can present predominantly with upper-airway disease and mimic croup or asthma. Although GPA is classically PR3-ANCA positive, MPO-ANCA positivity can occur and is associated with limited disease and subglottic stenosis. Multidisciplinary management and immunosuppression led to sustained symptom resolution and declining antibody titers.

Asset Subtitle

Radha Vyas

Meta Tag

Author List Catherine Chen, Radha Vyas

Category Clinical Vignettes

Concept Granulomatosis with Polyangiitis

Concept Subglottic Stenosis

Concept Upper Airway Symptoms

Concept Direct Laryngoscopy

Concept Immunosuppressive Therapy

Distinguished Non-Finalist

Presenter Organization Rutgers Robert Wood Johnson Medical School

Presenting Author Radha Vyas

Track Adult

Keywords

granulomatosis with polyangiitis

subglottic stenosis

upper airway disease

MPO-ANCA

p-ANCA

dysphonia

dyspnea

asthma mimic

tracheal dilation

nasal crusting

Granulomatosis with Polyangiitis

Subglottic Stenosis

Upper Airway Symptoms

Direct Laryngoscopy

Immunosuppressive Therapy