Thrombotic Microangiopathy Associated With Macrophage Activa
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A study was carried out to describe the clinical characteristics, treatment, and outcomes of a multinational cohort of patients with macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA).

Patients with MAS and TMA co-occurred were asked to obtain data retrospectively from international pediatric rheumatologists. Patients with chronic juvenile idiopathic arthritis (sJIA)-associated MAS and TMA were linked to a historical cohort of sJIA and MAS patients in terms of clinical and laboratory characteristics.

--23 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, sJIA patients 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 (LDH), bilirubin and D-dimer, and lower levels of albumin and fibrinogen.

--They also required admission to the ICU 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.

Finally, the possibility of MAS and TMA coexistence in rheumatic diseases may be underappreciated. Patients with MAS who develop disproportionate anemia, thrombocytopenia, or an increase in LDH, as well as those who have multi-organ failure, should be evaluated for this connection.