目的:我们旨在表征临床和放射学特征,和结果,来自单个中心的大量肥厚性硬脑膜炎(HP)患者。
方法:我们在三级转诊中心进行了一项回顾性研究,涵盖2003年至2022年间诊断为HP的患者。HP的诊断依赖于通过脑或脊柱的磁共振成像(MRI)识别硬膜增厚。
结果:我们纳入了74例患者,平均年龄为43.6±14.2岁,其中37人(50%)为男性。其中,32(43.2%)具有免疫介导的起源,包括21例肉芽肿性多血管炎(GPA)(主要是PR3-ANCA阳性),四个患有系统性红斑狼疮,三个与IgG4相关的疾病,三例特发性HP,还有一个患有类风湿性关节炎.非免疫介导的HP占45例(56.8%)。在这个类别中,感染病例21例(28.4%),14是结核分枝杆菌感染(TB-HP),恶性肿瘤相关HP21例(28.4%)。临床和MRI特征在四个病因组中表现出差异。低血糖主要在感染性和恶性肿瘤相关的HP中观察到。免疫介导的HP与对比增强的外周模式和埃菲尔夜间体征有关。强烈提示TB-HP的MRI特征包括软脑膜受累,脑实质病变,动脉中风.MPO-ANCAGPA与较高的脊髓HP患病率相关。
结论:在我们的队列中,GPA和结核分枝杆菌是HP的主要原因。我们发现不同病因在临床和放射学特征上存在显著差异,这可能对诊断有影响。
OBJECTIVE: We aimed to characterize the clinical and radiological features, and outcomes, of a large cohort of hypertrophic pachymeningitis (HP) patients from a single center.
METHODS: We conducted a retrospective study at a tertiary referral center, encompassing patients diagnosed with HP between 2003 and 2022. The diagnosis of HP relied on the identification of thickening of the dura mater via magnetic resonance imaging (MRI) of the brain or spine.
RESULTS: We included 74 patients with a mean age of 43.6 ± 14.2 years, of whom 37 (50%) were male. Among them, 32 (43.2%) had an immune-mediated origin, including 21 with granulomatosis with polyangiitis (GPA) (predominantly PR3-ANCA positive), four with systemic lupus erythematosus, three with IgG4-related disease, three with idiopathic HP, and one with rheumatoid arthritis. Non-immune-mediated HP accounted for 45 cases (56.8%). Within this category, 21 (28.4%) were infectious cases, with 14 being Mycobacterium tuberculosis infection (TB-HP), and 21 (28.4%) were malignancy-associated HP. Clinical and MRI characteristics exhibited variations among the four etiological groups. Hypoglycorrhachia was primarily observed in infectious and malignancy-associated HP. Immune-mediated HP was associated with a peripheral pattern of contrast enhancement and the Eiffel-by-night sign. MRI features strongly indicative of TB-HP included leptomeningeal involvement, brain parenchymal lesions, and arterial stroke. MPO-ANCA GPA was associated with a higher prevalence of spinal HP.
CONCLUSIONS: Within our cohort, GPA and Mycobacterium tuberculosis emerged as the predominant causes of HP. We identified significant disparities in clinical and radiological features among different etiologies, which could have implications for diagnosis.