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Thrombotic Microangiopathy Associated with Macrophage Activation Syndrome: A Multinational Study of 23 Patients
- Source :
- The Journal of pediatrics. 235
- Publication Year :
- 2020
-
Abstract
- 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.
- Subjects :
- musculoskeletal diseases
medicine.medical_specialty
Thrombotic microangiopathy
Adolescent
Thrombotic thrombocytopenic purpura
610 Medicine & health
typical hemolytic uremic syndrome
hemophagocytic lymphohistiocytosis
hemophagocytic syndromes
macrophage activation syndrome
thrombotic microangiopathy
thrombotic thrombocytopenic purpura
Antibodies, Monoclonal, Humanized
Gastroenterology
03 medical and health sciences
0302 clinical medicine
Mixed connective tissue disease
030225 pediatrics
Internal medicine
hemic and lymphatic diseases
Atypical hemolytic uremic syndrome
medicine
Humans
2735 Pediatrics, Perinatology and Child Health
030212 general & internal medicine
Child
Glucocorticoids
Juvenile dermatomyositis
Retrospective Studies
atypical hemolytic uremic syndrome
Plasma Exchange
business.industry
Thrombotic Microangiopathies
Macrophage Activation Syndrome
Undifferentiated connective tissue disease
Eculizumab
medicine.disease
Arthritis, Juvenile
10036 Medical Clinic
Macrophage activation syndrome
Antirheumatic Agents
Child, Preschool
Pediatrics, Perinatology and Child Health
business
Biomarkers
medicine.drug
Subjects
Details
- ISSN :
- 10976833
- Volume :
- 235
- Database :
- OpenAIRE
- Journal :
- The Journal of pediatrics
- Accession number :
- edsair.doi.dedup.....c8eca0af3953f7f145e41777b794b778