Rheumatology, Network Hospital Neuchâtel, La Chaux-de-Fonds, Switzerland
Rheumatology, Network Hospital Neuchâtel, La Chaux-de-Fonds, Switzerland.
RMD Open. 2022 Nov;8(2). doi: 10.1136/rmdopen-2022-002489.
The present case report focuses on an immunocompromised 81-year-old patient initially diagnosed with Waldenström's disease. The patient experienced a gradual vision loss and jaw pain with high erythrocyte sedimentation rate. We first suspected giant cell arteritis, despite inconclusive assessment, including a negative temporal artery biopsy. We rapidly started a corticosteroid pulse therapy followed by high-dose corticosteroid therapy that was followed even after discharge from the hospital. The patient was readmitted 20 days later with severe left retro-orbital pain and progressive left vision loss. Clinical examination revealed complete left eyelid ptosis and unilateral blindness with fixed mydriasis and no eye movement. MRI showed signs of ischaemic optic neuropathy with lysis of the left ethmoid sinus wall; thus, indicating ischaemic optic neuropathy related to lymphoplasmacytic infiltration of Waldenström's disease (Bing-Neel syndrome). Oncological treatment of ibrutinib, a tyrosine kinase inhibitor, was then administered. Despite a favourable prognosis, no improvement was seen. An infectious aetiology was finally confirmed. The left sphenoid sinus biopsy highlighted an angioinvasive aspergillosis with rhino-orbital infiltration observed as ischaemic optic neuropathy. Oncologic treatment was discontinued and antifungal therapy with voriconazole was introduced, leading to a favourable radiological development and analgesic control, without ophtalmological improvement.
本病例报告重点介绍了一位最初被诊断为华氏巨球蛋白血症的免疫功能低下 81 岁患者。该患者出现渐进性视力丧失和颌骨疼痛,红细胞沉降率升高。我们最初怀疑是巨细胞动脉炎,但评估结果不一致,包括颞动脉活检呈阴性。尽管如此,我们仍迅速开始了皮质类固醇脉冲治疗,随后进行了大剂量皮质类固醇治疗,即使在出院后也持续进行。20 天后,该患者因严重的左眼眶后疼痛和左眼视力逐渐丧失再次入院。临床检查显示左眼完全上睑下垂和单侧失明,伴有固定性瞳孔散大且无眼球运动。MRI 显示缺血性视神经病变的迹象,伴有左侧筛窦壁溶解;因此,提示与华氏巨球蛋白血症(Bing-Neel 综合征)相关的淋巴浆细胞浸润相关的缺血性视神经病变。随后给予伊布替尼,一种酪氨酸激酶抑制剂进行肿瘤治疗。尽管预后良好,但未见改善。最终确认了感染性病因。左侧蝶窦活检突出显示侵袭性血管内曲霉菌病,伴有鼻-眶侵袭,表现为缺血性视神经病变。停止了肿瘤治疗,引入了伏立康唑抗真菌治疗,导致影像学得到良好的发展和疼痛控制,但眼科无改善。