Thrombotic Microangiopathy Associated with Macrophage Activation Syndrome: A Multinational Study of 23 Patients.

Autor: Minoia F; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. Electronic address: francesca.minoia@policlinico.mi.it., Tibaldi J; IRCCS Istituto Giannina Gaslini, Genoa, Italy; Università degli Studi di Genova, Genoa, Italy., Muratore V; IRCCS Policlinico San Matteo, Pavia, Italy., Gallizzi R; Università degli Studi di Messina, Messina, Italy., Bracaglia C; IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy., Arduini A; IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy., Comak E; Akdeniz University, Antalya, Turkey., Vougiouka O; Kyriakou Hospital, Athens, Greece., Trauzeddel R; Helios Klinikum, Berlin, Germany., Filocamo G; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy., Mastrangelo A; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy., Micalizzi C; IRCCS Istituto Giannina Gaslini, Genoa, Italy., Kasapcopur O; Istanbul University-Cerrahpasa, Department of Pediatric Rheumatology, Istanbul, Turkey., Unsal E; Department of Pediatric Rheumatology, Dokuz Eylul University Childrens' Hospital, Izmir, Turkey., Kitoh T; Laboratory of Pediatrics, Aichi Gakuin University School of Pharmacy, Nagoya, Japan., Tsitsami E; 1st Department of Pediatrics, University of Athens, Children's Hospital Aghia Sophia, Athens, Greece., Kostik M; Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia., Schmid JP; Universitäts-Kinderspital, Zürich, Switzerland; University of Zurich, Zurich, Switzerland., Prader S; Universitäts-Kinderspital, Zürich, Switzerland., Laube G; Universitäts-Kinderspital, Zürich, Switzerland., Maritsi D; National and Kopdistrian University of Athens, Athens, Greece., Jelusic M; University of Zagreb School of Medicine, Zagreb, Croatia., Shenoi S; Seattle Children's Hospital, Seattle, WA, USA., Vastert S; University Medical Center Utrecht, Utrecht, the Netherlands., Ardissino G; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy., Cron RQ; Children's Hospital of Alabama, Birmingham, AL, USA., Ravelli A; IRCCS Istituto Giannina Gaslini, Genoa, Italy; Università degli Studi di Genova, Genoa, Italy; Sechenov First Moscow State Medical University, Moscow, Russian Federation.
Jazyk: angličtina
Zdroj: The Journal of pediatrics [J Pediatr] 2021 Aug; Vol. 235, pp. 196-202. Date of Electronic Publication: 2021 Apr 07.
DOI: 10.1016/j.jpeds.2021.04.004
Abstrakt: Objective: To describe the clinical characteristics, treatment, and outcomes of a multinational cohort of patients with macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA).
Study Design: International 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.
Results: Twenty-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.
Conclusions: The 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.)
Databáze: MEDLINE
Externí odkaz: