Article ; Online: Pediatric-onset limited ANCA-associated vasculitis arising during pre-existing chronic recurrent multifocal osteomyelitis.
Pediatric rheumatology online journal
2023 Volume 21, Issue 1, Page(s) 89
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 ... ...
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. |
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MeSH term(s) | Child ; Humans ; Pyoderma Gangrenosum ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy ; Osteomyelitis/complications ; Osteomyelitis/diagnosis ; Osteitis ; Anti-Inflammatory Agents, Non-Steroidal |
Chemical Substances | Anti-Inflammatory Agents, Non-Steroidal |
Language | English |
Publishing date | 2023-08-24 |
Publishing country | England |
Document type | Case Reports ; Journal Article |
ZDB-ID | 2279468-2 |
ISSN | 1546-0096 ; 1546-0096 |
ISSN (online) | 1546-0096 |
ISSN | 1546-0096 |
DOI | 10.1186/s12969-023-00876-x |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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