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A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts
Published on: September 20, 2018
Pediatric-onset limited ANCA-associated vasculitis arising during pre-existing chronic recurrent multifocal
Esraa Eloseily1,2, Michael Henrickson3
1Division of Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, 3333 Burnet avenue, Cincinnati, OH, 45229, USA. esraa.eloseily@cchmc.org.
Background:
Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by chronic vasculitis involving small to medium sized arteries, granulomatous inflammation of the upper and lower respiratory tracts, pauci-immune necrotizing glomerulonephritis, as well as vasculitis of other organs. Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory syndrome characterized by sterile bone inflammation.
Case Presentation:
We report a case of CRMO that was doing well on non-steroidal anti-inflammatory drugs (NSAID for 6 years and then developed ANCA positive limited GPA presenting with pyoderma gangrenosum, persistent bilateral otalgia with serous otitis, otorrhea, then sensorineural hearing loss.
Conclusion:
This is the first report of limited GPA initially presenting as pyoderma gangrenosum in a patient with underlying CRMO. It is unclear how the pathology of an autoimmune and an autoinflammatory condition can overlap.
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