Objective: To retrospectively analyze the effect of plasma exchange (PLEX; yes = PLEX+, no = PLEX–) and steroids administration timing (prophylactically [proST] or therapeutically [therST]) on the longitudinal clinical course of patients with natalizumab-related progressive multifocal leukoencephalopathy (PML) and full-blown immune reconstitution inflammatory syndrome (PML-IRIS). Methods: Clinical and radiological data of 42 Italian patients with PML were analyzed. Patient's data are available until 12 months after PML diagnosis. PLEX and steroids treatment as time-dependent covariates were entered in: (1) a Cox model to investigate their impact on full-blown PML-IRIS latency; (2) an analysis of variance ANOVA to investigate their impact on IRIS duration; and (3) a linear mixed model to assess their impact on the longitudinal clinical course (measured by means of Expanded Disability Status Scale [EDSS]). Results: Treatment with PLEX was not associated to PML-IRIS latency (hazard ratio [HR] = 1.05; p = 0.92), but once IRIS emerged, its duration was significantly longer in patients who underwent PLEX (101 vs 54 days in PLEX+ and PLEX– patients; p = 0.028). Receiving proST versus therST was not associated to IRIS latency (HR = 0.67; p = 0.39) or duration (p = 0.95). Patients who underwent proST had a significantly higher EDSS increase during PML (0.09 EDSS points per month; p = 0.04) as compared to those who had therST. Interpretation: This study highlights that: (1) caution on the use of PLEX should be considered as the current data do not support a beneficial effect of PLEX and (2) caution on the early use of steroids is suggested because their prophylactic use to prevent full-blown PML-IRIS seems to negatively impact on the longitudinal disability course. Ann Neurol 2017;82:697–705.

To do or not to do? plasma exchange and timing of steroid administration in progressive multifocal leukoencephalopathy

Artusi, Carlo Alberto;Bertolotto, Antonio;Capobianco, Marco;Cavaletti, Guido;Clerico, Marinella;Durelli, Luca;Laroni, Alice;Solaro, Claudio;Valentino, Paola
2017

Abstract

Objective: To retrospectively analyze the effect of plasma exchange (PLEX; yes = PLEX+, no = PLEX–) and steroids administration timing (prophylactically [proST] or therapeutically [therST]) on the longitudinal clinical course of patients with natalizumab-related progressive multifocal leukoencephalopathy (PML) and full-blown immune reconstitution inflammatory syndrome (PML-IRIS). Methods: Clinical and radiological data of 42 Italian patients with PML were analyzed. Patient's data are available until 12 months after PML diagnosis. PLEX and steroids treatment as time-dependent covariates were entered in: (1) a Cox model to investigate their impact on full-blown PML-IRIS latency; (2) an analysis of variance ANOVA to investigate their impact on IRIS duration; and (3) a linear mixed model to assess their impact on the longitudinal clinical course (measured by means of Expanded Disability Status Scale [EDSS]). Results: Treatment with PLEX was not associated to PML-IRIS latency (hazard ratio [HR] = 1.05; p = 0.92), but once IRIS emerged, its duration was significantly longer in patients who underwent PLEX (101 vs 54 days in PLEX+ and PLEX– patients; p = 0.028). Receiving proST versus therST was not associated to IRIS latency (HR = 0.67; p = 0.39) or duration (p = 0.95). Patients who underwent proST had a significantly higher EDSS increase during PML (0.09 EDSS points per month; p = 0.04) as compared to those who had therST. Interpretation: This study highlights that: (1) caution on the use of PLEX should be considered as the current data do not support a beneficial effect of PLEX and (2) caution on the early use of steroids is suggested because their prophylactic use to prevent full-blown PML-IRIS seems to negatively impact on the longitudinal disability course. Ann Neurol 2017;82:697–705.
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697
705
https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.25070
Adult; Databases, Factual; Disability Evaluation; Female; Humans; Immune Reconstitution Inflammatory Syndrome; Leukoencephalopathy, Progressive Multifocal; Male; Plasma Exchange; Retrospective Studies; Steroids; Young Adult
Scarpazza, Cristina; Prosperini, Luca; De Rossi, Nicola; Moiola, Lucia; Sormani, Maria Pia; Gerevini, Simonetta; Capra, Ruggero; Altieri, Marta; Amato, Maria Pia; Artusi, Carlo Alberto; Bandini, Fabio; Barcella, Valeria; Bertolotto, Antonio; Morra, Vincenzo Brescia; Capobianco, Marco; Cavaletti, Guido; Cavalla, Paola; Centonze, Diego; Clerico, Marinella; Cocco, Eleonora; Cordioli, Cinzia; Cosottini, Mirco; D'Aleo, Giangaetano; de Luca, Giovanna; de Riz, Marilena; Deotto, Luciano; Durelli, Luca; Falcini, Mario; Ferrari, Ernesta; Ferrante, Claudio; Fusco, Maria Luisa; Gasperini, Claudio; Ghezzi, Angelo; Grimaldi, Luigi; Guidotti, Mario; Laroni, Alice; Lugaresi, Alessandra; Naldi, Paola; Pane, Chiara; Perrone, Patrizia; Pizzorno, Matteo; Pozzilli, Carlo; Rezzonico, Monica; Rovaris, Marco; Salemi, Giuseppe; Salvetti, Marco; Santuccio, Giuseppe; Scarpini, Elio; Sessa, Edoardo; Solaro, Claudio; Tabiadon, Giulia; Tortorella, Carla; Trojano, Maria; Valentino, Paola
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2318/1758077
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