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Refractory otitis media: an unusual presentation of childhood granulomatosis with polyangiitis

Preena Uppal1, Jonny Taitz, Brynn Wainstein

  • 1Department of General Pediatrics, Sydney Children's Hospital Network, Randwick, Australia; School of Women's and Children's Health, University of New South Wales, Australia.

Pediatric Pulmonology
|January 30, 2013
PubMed

Insights

Childhood granulomatosis with polyangiitis (cGPA) is a rare vasculitis mimicking infection. Early diagnosis is crucial to prevent severe lung and hearing complications in children.

Area of Science:

  • Pediatric Rheumatology
  • Pulmonology
  • Otolaryngology

Background:

  • Childhood granulomatosis with polyangiitis (cGPA), formerly Wegener's granulomatosis, is a rare, life-threatening vasculitis.
  • cGPA symptoms can mimic infections, complicating early diagnosis in pediatric patients.

Observation:

  • A 16-year-old female presented with persistent middle ear effusion and progressive sensorineural hearing loss.
  • Following 6 months of treatment, she developed severe pneumonia with pleural effusion and cavitary lung lesions.

Findings:

  • Diagnostic investigations revealed high titers of cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA).
  • Nasal septal biopsy confirmed the diagnosis of cGPA.

Implications:

  • This case underscores the diagnostic challenges of cGPA in children.
  • Delayed diagnosis of cGPA can lead to severe, potentially fatal, multi-organ complications, including pulmonary and auditory damage.

Keywords:
Wegener's granulomatosisatypical pneumoniachildhood vasculitischronic otitis mediahearing loss

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