Refractory otitis media: an unusual presentation of childhood granulomatosis with polyangiitis.
Childhood granulomatosis with polyangiitis (cGPA), previously known as Wegener's granulomatosis, is a rare, potentially fatal necrotizing vasculitis, the symptoms of which overlap with infection. We present a 16-year-old girl who, following 6 months of treatment for persistent middle ear effusion with progressive sensorineural hearing loss, developed rapidly progressing pneumonia, with pleural effusion, and multiple cavitatory lung lesions. Investigations demonstrated high titer c-ANCA and nasal septal biopsy confirmed the diagnosis of cGPA. This case highlights the difficulty in diagnosing cGPA and the potentially life-threatening consequences of failing to do so.
Department of General Pediatrics, Sydney Children's Hospital Network, Randwick, Australia; School of Women's and Children's Health, University of New South Wales, Australia.
Preena
P
Uppal
Jonny
J
Taitz
Brynn
B
Wainstein
Marlene
M
Soma
Yvonne
Y
Belessis
Paul
P
Gray
D000293:Adolescent; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D002908:Chronic Disease; D003520:Cyclophosphamide / Q000627:therapeutic use; D003951:Diagnostic Errors; D004427:Ear Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D046089:Hearing Loss, Mixed Conductive-Sensorineural / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008171:Lung Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D008775:Methylprednisolone / Q000627:therapeutic use; D009668:Nose Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D010033:Otitis Media / Q000175:diagnosis*
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone
Wegener's granulomatosis; atypical pneumonia; childhood vasculitis; chronic otitis media; hearing loss