ANCA-associated refractory vasculitis with multiple systemic involvement: A rare case report.
Int J Rheum Dis
; 26(5): 965-967, 2023 May.
Article
en En
| MEDLINE
| ID: mdl-36599652
ABSTRACT
We report the case of a 65 year old female patient, presenting with a combination of bilateral hearing loss, otalgia, and hyperacusis. Pure tone audiometry revealed mixed bilateral hearing loss. Conventional cranial imaging tests failed to show a significant brain pathology, but fat-suppressed T1-weighted gadolinium-enhanced magnetic resonance imaging scan displayed a diffuse infiltrative skull base process, extending from the nasopharynx to the jugular fossa, and encasing the internal carotid artery. The latter findings, besides elevated inflammatory markers and a positive perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) led to the diagnosis of ANCA-associated vasculitis. Additional disease manifestations sequentially appeared, including a right peripheral nerve palsy, aortitis, hepatitis, peripheral neuropathy, and uveitis. Therapy with corticosteroids, azathioprine, and then cyclophosphamide brought no evident benefit, but rituximab led to impressive clinical and radiologic improvement.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Anticuerpos Anticitoplasma de Neutrófilos
/
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos
Tipo de estudio:
Diagnostic_studies
/
Risk_factors_studies
Límite:
Aged
/
Female
/
Humans
Idioma:
En
Revista:
Int J Rheum Dis
Asunto de la revista:
REUMATOLOGIA
Año:
2023
Tipo del documento:
Article
País de afiliación:
Israel