• Francesca Minoia, Jessica Tibaldi, Valentina Muratore, Romina Gallizzi, Claudia Bracaglia, Alessia Arduini, Elif Comak, Olga Vougiouka, Ralf Trauzeddel, Giovanni Filocamo, Antonio Mastrangelo, Concetta Micalizzi, Ozgur Kasapcopur, Erbil Unsal, Toshiyuki Kitoh, Elena Tsitsami, Mikhail Kostik, SchmidJana PachlopnikJPUniversitäts-Kinderspital, Zürich, Switzerland; University of Zurich, Zurich, Switzerland., Seraina Prader, Guido Laube, Despoina Maritsi, Marija Jelusic, Susan Shenoi, Sebastiaan Vastert, Gianluigi Ardissino, Randy Q Cron, Angelo Ravelli, and MAS/sJIA Working Group of the Pediatric Rheumatology European Society (PReS).
• Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. Electronic address: francesca.minoia@policlinico.mi.it.
• J. Pediatr. 2021 Aug 1; 235: 196-202.

ObjectiveTo describe the clinical characteristics, treatment, and outcomes of a multinational cohort of patients with macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA).Study DesignInternational pediatric rheumatologists were asked to collect retrospectively the data of patients with the co-occurrence of MAS and TMA. Clinical and laboratory features of patients with systemic juvenile idiopathic arthritis (sJIA)-associated MAS and TMA were compared with those of an historical cohort of patients with sJIA and MAS.ResultsTwenty-three patients with MAS and TMA were enrolled: 17 had sJIA, 2 systemic lupus erythematosus, 1 juvenile dermatomyositis, 1 mixed connective tissue disease, and 2 undifferentiated connective tissue disease. Compared with the historical cohort of MAS, patients with sJIA with coexistent MAS and TMA had higher frequencies of renal failure and neurologic involvement, hemorrhage, jaundice, and respiratory symptoms, as well as more severe anemia and thrombocytopenia, higher levels of alanine aminotransferase, lactate dehydrogenase, bilirubin and D-dimer, and lower levels of albumin and fibrinogen. They also required admission to the intensive care unit more frequently. Among patients tested, complement abnormalities and reduced ADAMTS13 activity were observed in 64.3% and 44.4% of cases, respectively. All patients received glucocorticoids. Treatment for TMA included plasma-exchange, eculizumab, and rituximab.ConclusionsThe possible coexistence of MAS and TMA in rheumatic diseases may be underrecognized. This association should be considered in patients with MAS who develop disproportionate anemia, thrombocytopenia, and lactate dehydrogenase increase, or have multiorgan failure.Copyright © 2021 Elsevier Inc. All rights reserved.

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