We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Pediatric-onset limited ANCA-associated vasculitis arising during pre-existing chronic recurrent multifocal osteomyelitis.
- Authors
Eloseily, Esraa; Henrickson, Michael
- Abstract
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.
- Subjects
GRANULOMATOSIS with polyangiitis; POLYARTERITIS nodosa; VASCULITIS; OSTEOMYELITIS; PYODERMA gangrenosum; OSTEITIS
- Publication
Pediatric Rheumatology, 2023, Vol 21, Issue 1, p1
- ISSN
1546-0096
- Publication type
Article
- DOI
10.1186/s12969-023-00876-x