Javed Arifa, Javed Sadia Arooj, Ostrov Barbara, Qian Jiang, Ngo Khoa
Albany Medical Center Department of Rheumatology, Albany, USA.
Punjab Medical College Department of Internal Medicine and Pediatrics, Faisalabad, Pakistan.
Case Rep Rheumatol. 2024 Aug 12;2024:2043012. doi: 10.1155/2024/2043012. eCollection 2024.
Giant cell arteritis (GCA) is a chronic granulomatous vasculitis of medium and large arteries leading to cranial and extracranial manifestations. Temporal artery biopsy is considered the gold standard; however, its sensitivity is low at 47%. We report a unique case of Bing-Neel Syndrome (BNS) presenting as biopsy-proven GCA. BNS is a rare complication (1%) of Waldenstrom Macroglobulinemia (WM), which results from infiltration of lymph plasmacytoid cells and plasma cells into the central nervous system. A 77-year-old female with a past medical history of glaucoma, hypertension, diabetes, and chronic ocular ischemic syndrome in her right eye presented with progressive left eye vision loss for 5 days. Fundoscopic examination was notable for pseudophakic pseudopallor but no optic disc edema. Intraocular pressure was >40 and normalized after acetazolamide. The patient was started on pulse dose steroids by her neuro-ophthalmologist. She was discharged home on 60 mg of prednisone. At follow up with her neuro-ophthalmologist, new dot blot hemorrhages in the left eye were noted and she was readmitted for pulse dose of intravenous methylprednisolone. Temporal artery biopsy was consistent with GCA spectrum. Work up revealed paraproteinemia and subsequent bone marrow biopsy demonstrated WM. The patient was treated for her WM and her ophthalmic complications stabilized.
巨细胞动脉炎（GCA）是一种累及中、大动脉的慢性肉芽肿性血管炎，可导致颅内和颅外表现。颞动脉活检被认为是金标准；然而，其敏感性较低，仅为47%。我们报告了一例独特的宾 - 尼尔综合征（BNS），表现为经活检证实的GCA。BNS是华氏巨球蛋白血症（WM）的一种罕见并发症（1%），由淋巴浆细胞样细胞和浆细胞浸润中枢神经系统所致。一名77岁女性，既往有青光眼、高血压、糖尿病病史，右眼患有慢性眼部缺血综合征，因左眼渐进性视力丧失5天就诊。眼底检查可见假晶状体性假性苍白，但无视盘水肿。眼压>40，使用乙酰唑胺后恢复正常。患者由神经眼科医生开始给予冲击剂量的类固醇治疗。出院时服用60毫克泼尼松。在随访神经眼科医生时，发现左眼出现新的点状出血，遂再次入院接受静脉注射甲泼尼龙冲击治疗。颞动脉活检结果符合GCA谱系。进一步检查发现副蛋白血症，随后骨髓活检证实为WM。患者接受了WM治疗，眼部并发症得到稳定。
