Τίτλος:
Thrombotic Microangiopathy Associated with Macrophage Activation
Syndrome: A Multinational Study of 23 Patients
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
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.
Συγγραφείς:
Minoia, Francesca
Tibaldi, Jessica
Muratore, Valentina and
Gallizzi, Romina
Bracaglia, Claudia
Arduini, Alessia
Comak,
Elif
Vougiouka, Olga
Trauzeddel, Ralf
Filocamo, Giovanni and
Mastrangelo, Antonio
Micalizzi, Concetta
Kasapcopur, Ozgur and
Unsal, Erbil
Kitoh, Toshiyuki
Tsitsami, Elena
Kostik,
Mikhail
Schmid, Jana Pachlopnik
Prader, Seraina
Laube, Guido
and Maritsi, Despoina
Jelusic, Marija
Shenoi, Susan
Vastert,
Sebastiaan
Ardissino, Gianluigi
Cron, Randy Q.
Ravelli,
Angelo
Pediat Rheumatology European Soc
Περιοδικό:
Journal of Pediatric Neurosciences
DOI:
10.1016/j.jpeds.2021.04.004
