结节病患者的进行性多灶性白质脑病。
Progressive multifocal leukoencephalopathy in patients with sarcoidosis.
机构信息
From the Service de Médecine Interne (Y.J., L.V., C. Bernard, C. Broussolle, P.S.), Hôpital de la Croix-Rousse, Lyon; Service de Médecine Interne (A.N., M.L.-D., M.H.) and Service de Neurologie (B.G.), CHU Nantes; Service d'Endocrinologie (A.F.), Diabète et Nutrition, CHU Reims; Biostatistic and Medical Information Department (S.K.), UMR 717 INSERM, Saint Louis University Hospital, AP-HP, University of Paris VII Denis Diderot, Paris; Assistance Publique-Hôpitaux de Paris (D.B., D.V.), Hôpital Avicenne et Université Paris 13, Sorbonne Paris Cité, Bobigny; Service de Médecine Interne et Gériatrie (C.R.), CH Belfort-Montbéliard, Belfort; Service de Pneumologie (S.D.), CHU Nicolle, Rouen; Service de Médecine Interne (M.R.), CH Oudot, Bourgoin-Jallieu; Department of Internal Medicine II (C.C.-A.), CHU Pitié Salpêtrière, Université Pierre et Marie Curie, Paris; and Service de Neuro-oncologie (F.D.), Hôpital Neurologique, Lyon, France.
出版信息
Neurology. 2014 Apr 15;82(15):1307-13. doi: 10.1212/WNL.0000000000000318. Epub 2014 Mar 7.
OBJECTIVE
To describe characteristics, risk factors, and treatment outcome of progressive multifocal leukoencephalopathy (PML) complicating sarcoidosis.
METHODS
A retrospective chart and literature review was performed. Patients were identified through records from physicians of the Groupe Sarcoïdose Francophone. Each case was compared with 3 controls.
RESULTS
Ten cases were found (8 men). The median age at sarcoidosis diagnosis was 34.9 (±6) years. PML and sarcoidosis were diagnosed concomitantly in 2 cases, while sarcoidosis was previously known in 8 cases, including 7 cases treated with steroids (mean time between sarcoidosis diagnosis and PML was 114 [±99] months). The mean CD4 cell count was 215 (±139)/mm(3). Neurosarcoidosis was thought to be the problem in 8 cases and treatment was intensified, delaying PML diagnosis by 4.5 (±3.9) months. Eight patients received PML-specific treatment. On the whole, 6 patients died of PML within a mean time of 8 (±4.3) months. Patients with PML were significantly younger than controls. When combining our 10 patients with another 20 from the literature, we found that 17 patients (57%) died from sarcoidosis-associated PML; thus, the fatality rate was 57%.
CONCLUSIONS
PML during sarcoidosis is often misdiagnosed. It is not associated with severe CD4 lymphocytopenia. Fatality rate is high in comparison with PML associated with other conditions. Interrupting immunosuppression remains the mainstay of treatment.
目的
描述并发于结节病的进行性多灶性白质脑病（PML）的特征、危险因素和治疗结果。
方法
进行了回顾性图表和文献复习。通过法国结节病组医生的记录确定患者。每个病例均与 3 个对照进行比较。
结果
发现 10 例（8 例男性）。结节病诊断时的中位年龄为 34.9（±6）岁。2 例同时诊断为 PML 和结节病，8 例先前已知结节病，其中 7 例接受类固醇治疗（从结节病诊断到 PML 的平均时间为 114（±99）个月）。平均 CD4 细胞计数为 215（±139）/mm3。8 例患者认为存在神经结节病，治疗得到强化，使 PML 诊断延迟了 4.5（±3.9）个月。8 例患者接受了 PML 特异性治疗。总体而言，6 例患者在平均 8（±4.3）个月内死于 PML。患有 PML 的患者明显比对照组年轻。当我们将 10 例患者与文献中的另外 20 例患者结合起来时，我们发现 17 例（57%）患者死于结节病相关性 PML；因此，死亡率为 57%。
结论
结节病期间的 PML 常被误诊。它与严重的 CD4 淋巴细胞减少无关。与其他疾病相关的 PML 相比，死亡率很高。中断免疫抑制仍然是治疗的主要方法。
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