Yvan Jamilloux1, Antoine Néel, Marie Lecouffe-Desprets, Anne Fèvre, Sebastien Kerever, Benoit Guillon, Diane Bouvry, Loig Varron, Cécile Redares, Stéphane Dominique, Mareille Roux, Catherine Chapelon-Abric, Dominique Valeyre, François Ducray, Claire Bernard, Christiane Broussolle, Mohamed Hamidou, Pascal Sève. 1. 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.
Abstract
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.
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 CD4lymphocytopenia. Fatality rate is high in comparison with PML associated with other conditions. Interrupting immunosuppression remains the mainstay of treatment.
Authors: Sina C Rosenkranz; Vivien Häußler; Manuela Kolster; Anne Willing; Jakob Matschke; Christoph Röcken; Klarissa Stürner; Frank Leypoldt; Eva Tolosa; Manuel A Friese Journal: Brain Commun Date: 2021-12-16
Authors: Huzaefah Syed; Christian Ascoli; Catharina Fm Linssen; Christen Vagts; Thomas Iden; Aamer Syed; Jordana Kron; Kelly Polly; David Perkins; Patricia W Finn; Richard Novak; Marjolein Drent; Robert Baughman; Nadera J Sweiss Journal: Sarcoidosis Vasc Diffuse Lung Dis Date: 2020-06-30 Impact factor: 0.670