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Thrombotic microangiopathy associated with macrophage activation syndrome: A multinational study of 23 patients (CROSBI ID 303625)

Prilog u časopisu | izvorni znanstveni rad | međunarodna recenzija

(MAS/sJIA Working Group of the Pediatric Rheumatology European Society (PReS)) Minoia, Francesca ; Tibaldi, Jessica ; Muratore, Valentina ; Gallizzi, Romina ; Bracaglia, Claudia ; Arduini, Alessia ; Comak, Elif ; Vougiouka, Olga ; Trauzeddel, Ralf ; Filocamo, Giovanni et al. Thrombotic microangiopathy associated with macrophage activation syndrome: A multinational study of 23 patients // Journal of pediatrics, 235 (2021), 196-202. doi: 10.1016/j.jpeds.2021.04.004

Podaci o odgovornosti

Minoia, Francesca ; Tibaldi, Jessica ; Muratore, Valentina ; Gallizzi, Romina ; Bracaglia, Claudia ; Arduini, Alessia ; Comak, Elif ; Vougiouka, Olga ; Trauzeddel, Ralf ; Filocamo, Giovanni ; Mastrangelo, Antonio ; Micalizzi, Concetta ; Kasapcopur, Ozgur ; Unsal, Erbil ; Kitoh, Toshiyuki ; Tsitsami, Elena ; Kostik, Mikhail ; Pachlopnik Schmid, Jana ; Prader, Seraina ; Laube, Guido ; Maritsi, Despoina ; Jelušić, Marija ; Shenoi, Susan ; Vastert, Sebastiaan ; Ardissino, Gianluigi ; Cron, Randy Q. ; Ravelli, Angello

MAS/sJIA Working Group of the Pediatric Rheumatology European Society (PReS)

engleski

Thrombotic microangiopathy associated with macrophage activation syndrome: A multinational study of 23 patients

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.

typical hemolytic uremic syndrome ; hemophagocytic lymphohistiocytosis ; hemophagocytic syndromes ; macrophage activation syndrome ; thrombotic microangiopathy ; thrombotic thrombocytopenic purpura

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Podaci o izdanju

235

2021.

196-202

objavljeno

0022-3476

1097-6833

10.1016/j.jpeds.2021.04.004

Povezanost rada

Kliničke medicinske znanosti

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