Department of Internal Medicine, Hedi Chaker Hospital, Sfax, Tunisia.
Rom J Ophthalmol. 2024 Apr-Jun;68(2):187-190. doi: 10.22336/rjo.2024.35.
This paper aimed to describe another form of aggressive limited Granulomatosis with polyangiitis (GPA) revealed by dacryoadenitis. We report an unusually limited GPA in a 48-year-old man presenting with bilateral proptosis. She had never presented kidney or pulmonary manifestations, but her disease was persistently active including oto-rhino-laryngological manifestations, dacryoadenitis, and neurological manifestations unresponsive to corticosteroids and immunosuppressors. Granulomatosis with polyangiitis (GPA) is an auto-immune inflammatory vasculitis. Involvement of lacrimal glands as the first presentation is uncommon. It is characterized by the development of granulomas. Patients with orbital mass without lacrimal gland involvement have a higher rate of systemic disease, a severe clinical course, and a higher rate of recurrences. A patient with dacryoadenitis seems to be with a good prognosis. Eye manifestations were significantly more common in patients with pachymeningitis. MPO-ANCA-positive pachymeningitis was more frequent in older female patients. PR3-ANCA-positive pachymeningitis had more severe neurological damage. Induction treatment consists of intravenous methylprednisolone (IV) associated with cyclophosphamide. Faced with dacryoadenitis, it is important to screen for ANCA-associated vasculitis. GPA = Granulomatosis with polyangiitis, ANCA = Antineutrophil Cytoplasmic Antibodies.
本文旨在描述一种以泪腺炎为表现的侵袭性局限性肉芽肿性多血管炎（GPA）。我们报告一例不典型局限性 GPA 患者，表现为双侧眼球突出。患者无肾脏或肺部表现，但疾病持续活动，包括耳-鼻-喉表现、泪腺炎和神经表现，对皮质类固醇和免疫抑制剂无反应。GPA 是一种自身免疫性炎症性血管炎。以泪腺炎为首发表现的情况并不常见。其特征为肉芽肿的形成。眼眶肿块而无泪腺受累的患者，全身性疾病发生率较高、临床病程较重、复发率较高。有泪腺炎的患者似乎预后较好。眼表现多见于硬脑膜炎患者。MPO-ANCA 阳性硬脑膜炎更常见于老年女性患者。PR3-ANCA 阳性硬脑膜炎的神经损伤更严重。诱导治疗包括静脉注射甲基强的松龙（IV）联合环磷酰胺。面对泪腺炎，筛查抗中性粒细胞胞浆抗体相关性血管炎很重要。GPA = 肉芽肿性多血管炎，ANCA = 抗中性粒细胞胞浆抗体。
