Pediatric-onset limited ANCA-associated vasculitis arising during pre-existing chronic recurrent multifocal osteomyelitis.

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

SUBMITTER: Eloseily E 

PROVIDER: S-EPMC10463712 | biostudies-literature | 2023 Aug

REPOSITORIES: biostudies-literature