ESPN 51th Annual Meeting

ESPN 2018


 
GRANULOMATOSIS WITH POLYANGIITIS PRESENTING AS MASTOIDITIS IN A CHILD
ENGIN MELEK 1 ÖZLEM ÖZGÜR GÜNDEŞLİOĞLU 1 AYSUN KARABAY BAYAZIT 1 EMİNE KOCABAŞ 1

1- CUKUROVA UNIVERSITY
 
Introduction:

 Granulomatosis with Polyangiitis (GPA) in childhood is a rare, severe, life-threatening disease. GPA in children frequently presents with upper-lower respiratory tract related symptoms or as Rapidly Progressive Glomerulonephritis (RPGN). In recent years, with advances in treatment protocols, despite significant improvement in the prognosis of the disease, the mortality and morbidity is still high. 

Material and methods:

 Here we report a 13.5-year-old female patient with the diagnosis of GPA presenting with chronic otitis media and mastoiditis and without pulmonary involvement. 

Results:

 From her history, we learned that befeore admission to our hospital, she admitted to another center with the complaint of ear pain and had the diagnosis of acute otitis media. Although received intensive antibiotic treatment; hearing loss, and fever have been added to her complaints. In her temporal CT, mastoiditis was diagnosed and although mastoidectomy and intensive antibiotic therapy, due to the lack of improvement, she admitted to our Pediatric Infectious Diseae Department. In our hospital, also bilateral mastoiditis and ethmoid sinusitis revealed and antibiotic therapy was started. On the fifth day of therapy, rise in serum creatinine was attributed to vancomycin toxicity because vancomycin level was very high. However, although the level of vancomycin was decreased in follow up, due to progressive increasing in creatinine level and also due to patient having hematuria, nephrotic-range proteinuria and treatment-resistant mastoiditis; RPGN secondary to ANCA associated vasculitis was thought and ANCA profiles were detected as positive and kidney biopsy compatible with vasculitis. In spite of the pulse steroids, plasmapheresis and cyclophosphamide therapies, due to lack of clinical and laboratory improvement, Rituximab was given to patient.  There were clinical improvement after rituximab and the patient is stable and the therapy is still ongoing. 

Conclusions:

This case is important to emphasize that in a case of mastoiditis which is resistant to treatment; vasculitis must come to mind. To our knowledge, this is the first child with GPA presenting with mastoiditis in the literature.