TY - JOUR
TI - Thrombotic Microangiopathy Associated with Macrophage Activation
Syndrome: A Multinational Study of 23 Patients
AU - Minoia, Francesca
AU - Tibaldi, Jessica
AU - Muratore, Valentina and
AU - Gallizzi, Romina
AU - Bracaglia, Claudia
AU - Arduini, Alessia
AU - Comak,
AU - Elif
AU - Vougiouka, Olga
AU - Trauzeddel, Ralf
AU - Filocamo, Giovanni and
AU - Mastrangelo, Antonio
AU - Micalizzi, Concetta
AU - Kasapcopur, Ozgur and
AU - Unsal, Erbil
AU - Kitoh, Toshiyuki
AU - Tsitsami, Elena
AU - Kostik,
AU - Mikhail
AU - Schmid, Jana Pachlopnik
AU - Prader, Seraina
AU - Laube, Guido
AU - and Maritsi, Despoina
AU - Jelusic, Marija
AU - Shenoi, Susan
AU - Vastert,
AU - Sebastiaan
AU - Ardissino, Gianluigi
AU - Cron, Randy Q.
AU - Ravelli,
AU - Angelo
AU - Pediat Rheumatology European Soc
JO - Journal of Pediatric Neurosciences
PY - 2021
VL - 235
TODO - null
SP - 196-202
PB - MOSBY-ELSEVIER
SN - 1817-1745
TODO - 10.1016/j.jpeds.2021.04.004
TODO - null
TODO - 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.
ER -