Division of Rheumatology, Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey.
Vasculitis Research Centre, Hacettepe University, Ankara, Turkey.
Int J Rheum Dis. 2023 Sep;26(9):1853-1860. doi: 10.1111/1756-185X.14725. Epub 2023 Jul 5.
Our understanding of IgG4-RD and pachymeningitis has grown substantially, but the optimal approach for diagnosis, management, and long-term outcomes is still an area of uncertainty.
HUVAC is a database for IgG4-RD patients, this database was retrospectively evaluated for pachymeningeal disease. Demographic, clinical, serological, imaging, histopathological data, and treatment details were re-interpreted in patients with pachymeningitis.
Among 97 patients with IgG4-RD, 6 (6.2%) had pachymeningitis. None of these patients had extracranial features, and also, in most of the patients, serum IgG4 levels were normal. Tentorium cerebelli and transverse sinus dura were the most commonly involved in the posterior fossa. During 18 months of median follow-up on steroid+-rituximab, none of them relapsed as pachymeningitis.
Our patients were mainly older males with sole neurological involvement. Non-specific headache was the most common manifestation, and serum IgG4 levels were not useful for diagnosis. Typical radiology and tentorial thickening should suggest IgG4-RD and prompt an early biopsy. Moreover, accompanying hypophysitis could also be a clue. With steroids+ rituximab treatment, no relapse related to meningeal involvement was seen in long-term follow-up.
我们对 IgG4-RD 和硬脑膜炎的认识有了很大的提高，但诊断、管理和长期预后的最佳方法仍存在不确定性。
HUVAC 是一个 IgG4-RD 患者数据库，本数据库对硬脑膜炎疾病进行了回顾性评估。对硬脑膜炎患者的人口统计学、临床、血清学、影像学、组织病理学数据和治疗细节进行重新解释。
在 97 例 IgG4-RD 患者中，有 6 例（6.2%）患有硬脑膜炎。这些患者均无颅外表现，且大多数患者血清 IgG4 水平正常。小脑幕和横窦硬脑膜是后颅窝最常受累的部位。在中位随访 18 个月的类固醇+-利妥昔单抗治疗期间，他们均未复发为硬脑膜炎。
我们的患者主要为老年男性，仅有神经系统受累。非特异性头痛是最常见的表现，血清 IgG4 水平对诊断无帮助。典型的影像学表现和小脑幕增厚应提示 IgG4-RD，并促使尽早进行活检。此外，伴随的垂体炎也可能是一个线索。采用类固醇+利妥昔单抗治疗，长期随访中未见脑膜受累相关复发。
