Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project

作者：Francesco Baldo, Remco Erkens, Mao Mizuta, Greta Rogani, Federica Lucioni, Claudia Bracaglia, Dirk Foell, Marco Gattorno, Marija Jelušić, Jordi Antón, Paul Brogan, Scott Canna, Shanmuganathan Chandrakasan, Randy Q. Cron, Fabrizio De Benedetti, Alexei A. Grom, Merav Heshin‐Bekenstein, AnnaCarin Horne, Raju Khubchandani, Seza Özen, Pierre Quartier, Angelo Ravelli, Masaki Shimizu, Grant S. Schulert, Christiaan Scott, Rashmi Sinha, Nicolino Ruperto, Joost F. Swart, Sebastiaan J. Vastert, Francesca Minoia, Kristiina Aalto, Carlos Abud‐Mendoza, Awatif Abushhaiwia, Constantin Ailioaie, Jonathan Akikusa, Güzide Aksu, Nuray Aktay Ayaz, Ruqaiya Nasser Al Jashmi, Safiya Al‐Abrawi, Maria Alessio, Ekaterina Alexeeva, Sulaiman M. Al‐Mayouf, Abdulaziz Almutairi, Muatasem Alsuweiti, Rizqi Amalia, Iman Amin, Jordi Antón, Wineke Armbrust, Itziar Astigarraga, Sevcan A. Bakkaloğlu, Reima Bakry, Özge Başaran, Floricely Basulto May, Jigna N Bathia, Cristina Battagliotti, Alexandre Bélot, Stefan Berg, Karin Beutel, Blanca Bica, Isabel Bolt, Martin Boyadzhiev, Oksana Boyarchuk, Yaryna Boyko, Claudia Bracaglia, Luciana Breda, Karine Brochard, Paul Brogan, Jürgen Brunner, Segundo Bujan Rivas, Aurelia Calin, Joan Calzada, Marisol Camacho Lovillo, Scott Canna, Elvira Cannizzaro, Roberta Caorsi, Raffaella Carlomagno, Marco Cattalini, Camilla Celani, Kwai Yu Winnie Chan, Sharat Chandra, Shanmuganathan Chandrakasan, Chong U Chang, Vyacheslav Chasnyk, Iryna Chyzheuskaya, Coziana Ciurtin, Daniel Clemente, Alexis‐Virgil Cochino, Alessandro Consolaro, Rita Consolini, Randy Q. Cron, Carolina Veras Pessoa Da Silva, Fabrizio De Benedetti, Carmen De Cunto, Arianna De Matteis, Lien De Somer, Fatma Dedeoğlu, Chellapandian Deepakbabu, Emanuela Del Giudice, Adriana Soraya Diaz Maldonado, Pavla Doležalová, Frank Dressler, Marta Dzhus, Yasser El Miedany, Dalia El‐Ghoneimy, Wolfgang Emminger, Sandra Enciso, A Estmann, Hala Etayari, Danielle C. Fair, Maria Faßhauer, Daniel El Fassi, Enrique Faugier Fuentes, Silvia Federici, Brian M. Feldman, Giovanni Filocamo, Berit Flatø, Mark Fluchel, Dirk Foell, Lampros Fotis, Marijan Frković, Robert C. Fuhlbrigge, Carla Gaggiano, Alenka Gagro, Romina Gallizzi, Ana Luíza Garcia Cunha, Fernando García Rodríguez, Fernando García Rodríguez, Marco Gattorno, Hermann Girschick, Mia Glerup, Lyudmila Grebenkina, Suparna Guha, Raúl Gutiérrez Suárez, Jaime Guzman Ramirez, Djohra Hadef, Mohamad Hamad Saied, Soad Hashad, Philip J. Hashkes, Henrik Hasle, J. Allyson Hays, Martina Held, Jan‐Inge Henter, Cristina Herrera, Merav Heshin‐Bekenstein, Andrew Ho, AnnaCarin Horne, Gerd Horneff, Jing‐Long Huang, Markus Hufnagel, Jaanika Ilisson, Mohammad Imnul Islam, В.Н. Иванова, Kazushi Izawa, Aleš Janda, Marija Jelusic, Dae-Chul Jeong, Rohith Jesudas, Ankur Kumar Jindal, Karla Vanessa Jiron Mendiola, Sheila Knupp Feitosa de Oliveira, Robin Kahn, Rita Káposzta, Ozgur Kasapcopur, María Martha Katsicas, Akhila Kavirayani, Camille Keenan, Parichat Khaosut, Khulood Khawaja, Waleed Ahmed Salaheldeen Hassan Khedr, Raju Khubchandani, Katarzyna Kobusińska, Oya Köker, Isabelle Koné-Paut, Mikhail M. Kostik, Jasmin Kuemmerle‐Deschner, Ashish Kumar, Paul La Rosée, M. A. Ramírez, Călin Lazăr, Chongwei Lee, Hartwig Lehmann, Kai Lehmberg, Caifeng Li, Xiaoqing Li, Francesco Licciardi, Joana Esteves de Lima, Zoref Lorenz, Hala Lotfy, Daniel J. Lovell, Meiping Lu, Kristīne Lukjanoviča, Maria Cristina Maggio, Silvia Magni‐Manzoni, Sheren esam maher Maher, Mahmoud Majeed, Despoina Maritsi, Rebecca Marsh, Giorgia Martini, Tania Nicole Masmas, Maria Vincenza Mastrolia, Angela Mauro, F Mehregan, Manel Mejbri, Isabelle Melki, Päivi Miettunen, Angela Miniaci, Francesca Minoia, Mao Mizuta, Rakesh Mondal, Davide Montin, Zoilo Morel Ayala, Juan Manuel Mosquera Angarita, Zaure Mukusheva, Charlotte Myrup, EKA NAKHUTSRISHVILI, Ahmed Naqvi, Hidehiko Narazaki, Joao Nascimento, Carmen Navarrete, Ellen Nordal, Ekemini A. Ogbu, Benson Ogunjimi, Lawrence Okong’o, Filipa Oliveira‐Ramos, Alessia Omenetti, Violetta Opoka‐Winiarska, Francesca Orlando, Sümeyra Özdemir Çiçek, Seza Özen, Clare Pain, Priyankar Pal, Natalia Palmou‐Fontana, Charalampia Papadopoulou, Manuela Pardeo, Gordana Petrović, Mercedes Picarelli, Rakesh Kumar Pilania, Clarissa Pilkington, M. Pinedo, Polyxeni Pratsidou‐Gertsi, Chris Pruunsild, Maa‐Ohui Quarmyne, Pierre Quartier, Seyed Reza Raees Karami, Kim Ramme, Angelo Ravelli, Jérôme Razanamahery, Katariina Rebane, Agustín Remesal, K. Retornaz, Donato Rigante, Joseph M. Rocco, Adriana Rodrigues Fonseca, A Lozano, Sandra Rodriguez-Aguayo, Jorge Rojas, Martina Rossano, Samppa Ryhanen, Cláudia Saad Magalhães, Payman Sadeghi, Erdal Sağ, Blachy J. Dávila Saldaña, Farhad Salehzadeh, Judith Sánchez-Manubens, Sujata Sawhney, Grant S. Schulert, Adrien Schvartz, Yuksel Selcuk, Velma Mulaosmanović, Ethan S Sen, Seher Şener, Rachna Shanbhag Mohite, Avinash Sharma, Rawia Salama Shehata, Masaki Shimizu, Gabriele Simonini, Surjit Singh, Roubini Smerla, Aušra Šnipaitienė, Ali Sobh, Betül Sözeri, Mihaela Spârchez, Saša Sršen, Valda Staņēvicha, Joost F. Swart, Flavio Sztajnbok, Sirikarn Tangcheewinsirikul, Katya Temelkova, Klaus Tenbrock, Nataša Toplak, Lilibeth Torno, Matteo Trevisan, Maria Tsinti, Elena Tsitsami, Marinka Twilt, J. Merlijn van den Berg, Jan A. M. van Laar, Camilo Andres Vargas Rincon, Giulia Varnier, Sebastiaan J. Vastert, Gabriel Vega‐Cornejo, Lucio Verdoni, Diego Oscar Viola, Jelena Vojinović, Yulia Vyzhga, Björn E. Wahlin, Hiroyuki Wakiguchi, Peter Weiser, Ewa Więsik–Szewczyk, Kazuko Yamazaki, Junko Yasumura, Wei Yin, Huasong Zeng, Wei Zhang, Vahid Ziaee, Amit Ziv, Zbigniew Żuber · 发表于：Lara D. Veeken · 年份：2024 · DOI：10.1093/rheumatology/keae391 · 被引用次数：36 · 研究领域：Autoimmune and Inflammatory Disorders Research、Kawasaki Disease and Coronary Complications、Vitamin C and Antioxidants Research

OBJECTIVE: To assess current treatment in macrophage activation syndrome (MAS) worldwide and to highlight any areas of major heterogeneity of practice. METHODS: A systematic literature search was performed in both EMBASE and PubMed databases. Paper screening was done by two independent teams based on agreed criteria. Data extraction was standardized following the PICO framework. A panel of experts assessed paper validity, using the Joanna Briggs Institute appraisal tools and category of evidence (CoE) according to EULAR procedure. RESULTS: Fifty-seven papers were finally included (80% retrospective case-series), describing 1148 patients with MAS: 889 systemic juvenile idiopathic arthritis (sJIA), 137 systemic lupus erythematosus (SLE), 69 Kawasaki disease (KD) and 53 other rheumatological conditions. Fourteen and 11 studies specified data on MAS associated to SLE and KD, respectively. All papers mentioned glucocorticoids (GCs), mostly methylprednisolone and prednisolone (90%); dexamethasone was used in 7% of patients. Ciclosporin was reported in a wide range of patients according to different cohorts. Anakinra was used in 179 MAS patients, with a favourable outcome in 83% of sJIA-MAS. Etoposide was described by 11 studies, mainly as part of HLH-94/04 protocol. Emapalumab was the only medication tested in a clinical trial in 14 sJIA-MAS, with 93% of MAS remission. Ruxolitinib was the most reported Janus kinase inhibitor in MAS. CONCLUSION: High-dose GCs together with IL-1 and ...