THU0530 Macrophage Activation Syndrome in Patients with Rheumatic Diseases – Single Center Experience
Authors: S. Rodionovskaya, I. Nikishina, I. Alakaeva, M. Kaleda
Journal: Annals of the Rheumatic Diseases (2015)

<h3>Background</h3> Macrophage activation syndrome (MAS) is a potentially fatal and hard to manage complication of rheumatic diseases (RD). MAS early diagnosis is a real challenge, whiles timely and adequate therapy has the paramount importance for reduction of associated mortality. <h3>Objectives</h3> To analyze clinical and laboratory parameters, therapeutic regimens and MAS outcomes in pediatric patients with RD. <h3>Methods</h3> Retrospective analysis of 35 RD and MAS patients in the last 5 years. <h3>Results</h3> MAS was diagnosed in 35 pediatric RD pts (17girls/18boys) during the period of 2010-2014y: in 25 with sJIA, 5 - SLE, 2 - JDM, 1 overlap-syndrome, 2 Kawasaki disease. Mean age at MAS diagnosis was 7.9±4.6y, average disease duration 3,8 (1,0; 6,5) y, in 57,2% pts MAS manifestation accompanied the disease onset. Max. interval between the onset of the disease and MAS development was 12 years. Subclinical MAS was diagnosed in 32% sJIA pts; fulminant course, requiring intensive care in 31%. Most common MAS clinical manifestations were: febrile fever 100%, hepatomegaly 88%, splenomegaly 75%, CNS lesion 51%, coagulopathy 40%, lymphadenopathy 37%, respiratory distress syndrome 37%, carditis 28%, hemorrhagic rash 26%, urticaria 20%. In 3 pts MAS manifested with hepatitis complicated by acute liver failure. Among common MAS laboratory markers the following should be mentioned: hyperferritinemia 82% (&lt;1000μkg/l – 31%, 1000-10.000μkg/l – 18%, &gt;10.000μkg/l – 27%), ← transaminases LDH 82%, hypertriglyceridemia 68%, thrombocytopenia 63%, cytopenia 40%, DIC – syndrome (hypofibrinogenemia, ←D-dimers) – 37%, bone marrow haemophagocytosis was confirmed in 22/35 pts 63%. Most severe MAS course was documented in pts with ferritine levels &gt;10000μkg/l. Low NK-cell count was diagnosed in 36% cases. MAS therapy included: GCs in 100% of pts (methylprednisolone pulse-therapy 74%, dexamethasone 37%), IVIGs 86%, cyclosporine A (CsA) 31%, etoposide 2,8%. In 6 sJIA pts with GCs, IVIG, and CsA failure tocilizumab (Tcz) 10-12 mg/kg once in 14 days was initiated at Week 4, with subsequent MAS resolution documented at Weeks 2-4 after initiation of therapy. In 4 sJIA pts developing MAS at different time points in the course of the disease (from 1 to 5 years) Tcz was administered following the outbreak of sJIA flare; kanakinumab was administered in 1 patient, rituximab – in 4 SLE pts. There were 5 deaths registered (3 sJIA, 2 SLE) on days 1 through 257 from MAS onset. <h3>Conclusions</h3> RD – associated MAS is most common in sJIA patients (71%), with subclinical course in 1/3 of all cases. Our data indicates that CNS involvement is the most common MAS sign. Fulminant MAS is associated with liver failure and respiratory distress-syndrome. We9ve accumulated positive experience with successful use of biological agents in patients with secondary MAS (Tcz, rituximab, kanakinumab). <h3>References</h3> Davì S, Consolaro A, et al. An international consensus survey of diagnostic criteria for macrophage activation syndrome in sJIA. Journal of Rheumatology.2011;38(4):764–768. Miettunen PM, Narendran A, et al. Successful treatment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition following conventional immunosuppressive therapy: case series with 12 patients. Rheumatology 2011;50:417–9 <h3>Disclosure of Interest</h3> None declared