DOI: 10.36347/sjmcr.2019.v07i05.007

Pages: 333–335

A 77-year-old woman presented with right visual disturbance. Her past medical history included left hearing loss and body weight loss. On ophthalmic examination, best-corrected visual acuity was counting finger in the right eye and 1.2 in the left eye. Brain magnetic resonance imaging (MRI) demonstrated right optic perineuritis and left mastoiditis. Laboratory examination revealed elevated erythrocyte sedimentation rate, C-reactive protein (CRP), and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). Urinalysis revealed a microscopic hematuria. Therefore, she received a diagnosis of microscopic polyangiitis (MPA) concomitant with IgG4-related disease (IgG4-RD). The patient was treated for oral prednisolone. After treatment, the symptoms showed gradually improved and her visual acuity improved to 0.01 in the right eye. MPO-ANCA and CRP were gradually improved, and the MRI findings of optic perineuritis and mastoiditis were also improved. This case highlights the importance for clinicians to be aware of a concomitant diagnosis of MPA and IgG4-RD.