From the Division of Neuroimmunology and Neuroinfectious Diseases (S.K.H., N.V.), Department of Neurology, Massachusetts General Hospital, Boston; Division of Neuroradiology (M.D.M), Department of Radiology, Massachusetts General Hospital, Boston; and Division of Rheumatology, Allergy and Immunology (E.M.M), Department of Medicine, Massachusetts General Hospital, Boston.
Neurol Neuroimmunol Neuroinflamm. 2021 Jun 2;8(5). doi: 10.1212/NXI.0000000000001022. Print 2021 Jul.
To review the previous literature on the associations of pachymeningitis with Crohn disease (CD) and relapsing polychondritis (RP) and to describe a new case occurring in association with both in addition to highlighting its positive response to steroid and adalimumab treatment.
We review the patient's clinical presentation, diagnostic workup (serum and CSF testing), and MRI findings in detail and chronicle the response of the pachymeningitis to intensive immunotherapy. We contrast this case against previous reports of pachymeningitis occurring in association with RP and inflammatory bowel disease that were found on PubMed.
Only 2 cases of ulcerative colitis and 5 cases of RP were found in association with pachymeningitis; there were no cases in association with CD. Our patient presented with symptoms isolated to a steroid-responsive headache in the setting of normal neurologic and rheumatologic examinations. Her preceding history was notable for long-standing CD and increasingly active symptoms referable to RP. Focal nodular pachymeningitis was seen overlying the left hemisphere on brain MRI. An extensive serum and CSF workup and body fluorodeoxyglucose-PET scan failed to identify an alternative etiology beyond her underlying autoimmune inflammatory disorders. After adding prednisone and adalimumab to her preexisting treatment of methotrexate, she responded dramatically both clinically and radiographically.
Although exceptionally rare, pachymeningitis may occur as a neuroinflammatory complication of CD and RP.
回顾先前关于硬脑膜炎与克罗恩病（CD）和复发性多软骨炎（RP）关联的文献，并描述一例新病例，该病例同时伴有这两种疾病，并强调其对类固醇和阿达木单抗治疗的积极反应。
我们详细回顾了患者的临床表现、诊断检查（血清和 CSF 检测）和 MRI 结果，并记录了硬脑膜炎对强化免疫治疗的反应。我们将该病例与在 PubMed 上找到的先前报道的与 RP 和炎症性肠病相关的硬脑膜炎病例进行了对比。
仅发现 2 例溃疡性结肠炎和 5 例 RP 与硬脑膜炎相关；没有与 CD 相关的病例。我们的患者表现为孤立性的类固醇反应性头痛，同时伴有正常的神经和风湿病学检查。她的既往病史有长期的 CD 和越来越活跃的 RP 症状。脑部 MRI 显示左半球上方有局灶性结节性硬脑膜炎。广泛的血清和 CSF 检查以及全身氟脱氧葡萄糖-PET 扫描未能发现除潜在自身免疫性炎症性疾病以外的其他病因。在将泼尼松和阿达木单抗加入她现有的甲氨蝶呤治疗后，她在临床和影像学上都有了显著的反应。
尽管非常罕见，但硬脑膜炎可能是 CD 和 RP 的神经炎症并发症。
