Guzman Samuel, Kleinschmidt-DeMasters B K
Department of Pathology, University of Colorado Health Science Center, Anschutz Medical Campus, Aurora, CO, USA.
Department of Pathology, University of Colorado Health Science Center, Anschutz Medical Campus, Aurora, CO, USA; Department of Neurosurgery, University of Colorado Health Science Center, Anschutz Medical Campus, Aurora, CO, USA; Department of Neurology, University of Colorado Health Science Center, Anschutz Medical Campus, Aurora, CO, USA.
Ann Diagn Pathol. 2025 Feb;74:152412. doi: 10.1016/j.anndiagpath.2024.152412. Epub 2024 Nov 22.
Rheumatoid meningitis (RM) presents with sufficiently wide-ranging, but non-specific, symptoms and neuroimaging features of pachy- and/or leptomeningeal thickening that it may be indistinguishable from subacute infectious meningitis. RA diagnosis variably antedates RM and serological confirmation by rheumatoid factor and anti-citrullinated peptide antibodies may not be present preoperatively. Thus, meningeal biopsy may be undertaken. Classic examples of RM show lymphoplasmacytic leptomeningeal inflammation and small vessel vasculitis, with rheumatoid nodules being less frequent. We reviewed our experience with 5 RM biopsies, as well as the literature, placing "classic" histological findings in perspective with biopsies showing less pathognomonic features. 5 RM cases were identified, 2 male: 3 female, ages 62-79 years. All patients had leptomeningeal enhancement by MRI and 2 had known negative RF serology prior to meningeal biopsy. In 1 case RF was initially negative, but on serological reassessment turned positive; 2 patients were diagnosed by clinical correlation. 4 leptomeningeal/superficial cortical biopsies manifested chronic lymphoplasmacytic inflammation with multinucleated giant cells, with discrete foci of deep blue/black necrosis with cellular debris ("dirty necrosis") surrounded by a variably- developed palisade of histiocytes (rheumatoid nodules). The 5th showed only non-specific mononuclear cell inflammation. All showed variable degrees of diffuse astrocytosis and microgliosis of the cortex without microglial clusters or compact granulomas. Stains for microorganisms were negative. Diagnosis of RM can be suspected by the pathologist if the "classic" features of rheumatoid nodules are present on biopsy, but in some cases, only non-specific inflammation is present. Diagnosis necessitates correlation between clinical, serological, and histological features.
类风湿性脑膜炎（RM）的症状和影像学特征广泛但不具特异性，表现为硬脑膜和/或软脑膜增厚，可能与亚急性感染性脑膜炎难以区分。类风湿性关节炎（RA）的诊断时间早于RM，术前可能无法通过类风湿因子和抗瓜氨酸化肽抗体进行血清学确诊。因此，可能需要进行脑膜活检。RM的典型病例表现为淋巴细胞浆细胞性软脑膜炎和小血管血管炎，类风湿结节较少见。我们回顾了5例RM活检的经验以及相关文献，将“典型”组织学表现与显示较少特征性表现的活检结果进行对比分析。共确定了5例RM病例，其中男性2例，女性3例，年龄在62至79岁之间。所有患者通过MRI检查均显示软脑膜强化，2例在脑膜活检前已知类风湿因子血清学检查结果为阴性。1例患者最初类风湿因子为阴性，但在血清学复查时转为阳性；2例患者通过临床相关性诊断。4例软脑膜/浅表皮质活检显示慢性淋巴细胞浆细胞性炎症伴多核巨细胞，有离散的深蓝色/黑色坏死灶，伴有细胞碎片（“污秽坏死”），周围有不同程度发育的组织细胞栅栏（类风湿结节）。第5例仅显示非特异性单核细胞炎症。所有病例均显示皮质有不同程度的弥漫性星形细胞增生和小胶质细胞增生，无小胶质细胞簇或致密肉芽肿。微生物染色均为阴性。如果活检显示有类风湿结节的“典型”特征，病理学家可怀疑为RM，但在某些情况下，仅存在非特异性炎症。诊断需要临床、血清学和组织学特征之间的相互关联。
