脊髓结节病的预后因素及治疗效果：一项长期随访的观察性队列研究
Prognostic Factors and Treatment Efficacy in Spinal Cord Sarcoidosis: An Observational Cohort With Long-term Follow-up.
作者信息
Gavoille Antoine, Desbois Anne-Claire, Joubert Bastien, Durel Cécile-Audrey, Auvens Clément, Berthoux Emilie, Delboy Thierry, Dufour Jean François, Turcu Alin, Bonnotte Bernard, Moreau Thibault, Le Guenno Guillaume, André Marc, Ruivard Marc, Camdessanche Jean-Philippe, Antoine Jean-Christophe G, Marignier Romain, Chapelon-Abric Catherine, Saadoun David, Sève Pascal
机构信息
From the Service de Neurologie, Sclérose en Plaques, Pathologies de la Myéline et Neuro-inflammation (A.G., R.M.) and Service de Neuro-Oncologie (B.J.), Hôpital Neurologique Pierre-Wertheimer, Hospices Civils de Lyon, Bron; Service de Biostatistique-Bioinformatique (A.G.), Département de Médecine Interne et Immunologie Clinique, Hôpital Édouard Herriot (C.-A.D.), and Département de Médecine Interne, Hôpital de la Croix Rousse (P.S.), Hospices Civils de Lyon; Université Lyon 1 (A.G., B.J., R.M.), Université de Lyon, Villeurbanne; Pitié-Salpêtrière University Hospital (A.-C.D., C.C.-A., D.S.), Sorbonne Universités; Department of Internal Medicine and Clinical Immunology (A.-C.D., C.C.-A., D.S.), AP-HP; Centre de Référence des Maladies Auto-Immunes Systémiques Rares (A.-C.D., C.C.-A., D.S.), Centre de Référence des Maladies Auto-Inflammatoires et de l'Amylose Inflammatoire, Paris; Département de Médecine Interne et Maladies Systémiques (C.A., A.T., B.B.) and Département de Neurologie (T.M.), CHU Dijon; Département de Médecine Interne (E.B.), CH Saint Luc Saint Joseph, Lyon; Département de Médecine Interne (T.D.), CH Montluçon; Département de Médecine Interne (J.F.D.), Centre Hospitalier Fleyriat, Bourg-en-Bresse; Département de Médecine Interne, CHU Estaing (G.L.G., M.R.), and Service de Médecine Interne, Hôpital Gabriel Montpied (M.A.), CHU de Clermont-Ferrand; Département de Neurologie (J.-P.C., J.-C.G.A.), Hôpital Nord, CHU de Saint-Etienne; and Research on Healthcare Performance (RESHAPE) (P.S.), INSERM U1290, Université Claude Bernard Lyon 1, France.
出版信息
Neurology. 2022 Apr 5;98(14):e1479-e1488. doi: 10.1212/WNL.0000000000200020. Epub 2022 Feb 10.
BACKGROUND AND OBJECTIVES
Spinal cord sarcoidosis is a rare manifestation of sarcoidosis with a consequent risk of neurologic sequelae for the patient. We investigated prognostic factors and efficacy of immunosuppressive treatments in a longitudinal cohort.
METHODS
We retrospectively studied patients with spinal cord sarcoidosis followed between 1995 and 2021 in 7 centers in France. Patients with definite, probable, or possible spinal cord sarcoidosis according to the Neurosarcoidosis Consortium Consensus Group criteria and with spinal cord involvement confirmed by MRI were included. We analyzed relapse or progression rate with a Poisson model, initial Rankin score with a linear model, and change in the Rankin score during follow-up with a logistic model.
RESULTS
A total of 97 patients were followed for a median of 7.8 years. Overall mean relapse or progression rate was 0.17 per person-year and decreased over time. At last visit, 46 (47.4%) patients had a loss of autonomy (Rankin score ≥2). The main prognostic factors significantly associated with relapse or progression rate were gadolinium enhancement (relative rate [95% CI] 0.61 [0.4, 0.95]) or meningeal involvement (relative rate [95% CI] 2.05 [1.31, 3.19]) on spinal cord MRI and cell count (relative rate [95% CI] per 1 log increase 1.16 [1.01, 1.33]) on CSF analysis. Relapse or progression rate was not significantly associated with initial Rankin score or Expanded Disability Status Scale. Tumor necrosis factor-α (TNF-α) antagonists significantly decreased relapse or progression rate compared with corticosteroids alone (relative rate [95% CI] 0.33 [0.11, 0.98]). Azathioprine was significantly less effective than methotrexate on relapse or progression rate (relative rate [95% CI] 2.83 [1.04, 7.75]) and change in Rankin score (mean difference [95% CI] 0.65 [0.23, 1.08]).
DISCUSSION
Regarding the relapse or progression rate, meningeal localization of sarcoidosis was associated with a worse prognosis, TNF-α antagonists resulted in a significant decrease compared to corticosteroids alone, and methotrexate was more effective than azathioprine.
CLASSIFICATION OF EVIDENCE
This study provides Class IV evidence that in individuals with spinal cord neurosarcoidosis, TNF-α antagonists were associated with decreased relapse or progression rate compared to corticosteroids alone, but other therapies showed no significant benefit.
背景与目的
脊髓结节病是结节病的一种罕见表现形式，会给患者带来神经后遗症风险。我们在一个纵向队列中研究了免疫抑制治疗的预后因素及疗效。
方法
我们回顾性研究了1995年至2021年期间在法国7个中心接受随访的脊髓结节病患者。纳入符合神经结节病协会共识小组标准的确诊、可能或疑似脊髓结节病且MRI证实有脊髓受累的患者。我们用泊松模型分析复发或进展率，用线性模型分析初始Rankin评分，用逻辑模型分析随访期间Rankin评分的变化。
结果
共对97例患者进行了中位时间为7.8年的随访。总体平均复发或进展率为每人年0.17，并随时间下降。在最后一次随访时，46例（47.4%）患者出现自主能力丧失（Rankin评分≥2）。与复发或进展率显著相关的主要预后因素是脊髓MRI上的钆增强（相对率[95%CI]0.61[0.4,0.95]）或脑膜受累（相对率[95%CI]2.05[1.31,3.19]）以及脑脊液分析中的细胞计数（每增加1个对数的相对率[95%CI]1.16[1.01,1.33]）。复发或进展率与初始Rankin评分或扩展残疾状态量表无显著关联。与单独使用皮质类固醇相比，肿瘤坏死因子-α（TNF-α）拮抗剂显著降低了复发或进展率（相对率[95%CI]0.33[0.11,0.98]）。在复发或进展率以及Rankin评分变化方面，硫唑嘌呤的疗效明显低于甲氨蝶呤（相对率[95%CI]2.83[1.04,7.75]）和平均差异[95%CI]0.65[0.23,1.08]）。
讨论
关于复发或进展率，结节病的脑膜定位与预后较差相关，TNF-α拮抗剂与单独使用皮质类固醇相比导致显著下降，且甲氨蝶呤比硫唑嘌呤更有效。
证据分级
本研究提供了IV级证据，即在脊髓神经结节病患者中，与单独使用皮质类固醇相比，TNF-α拮抗剂与复发或进展率降低相关，但其他疗法未显示出显著益处。
Zotero 插件