scholarly journals AB0725 MACROPHAGE ACTIVATION SYNDROME IN CHILDREN WITH RHEUMATIC DISEASES: ANALYSIS OF CASE SERIES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1393.3-1394
Author(s):  
M. Kaleda ◽  
I. Nikishina ◽  
V. Matkava ◽  
A. Shapovalenko ◽  
E. Fedorov ◽  
...  
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Early Stage ◽  
Clinical Manifestations ◽  
Case Series ◽  
Overlap Syndrome ◽  
High Dose ◽  
Female Ratio ◽  
Course Of Disease ◽  
Activation Syndrome

Background:Macrophage activation syndrome (MAS) is a rare, but severe life-threatening complication of chronic rheumatic disease (RD) in children, which associated with high risks of the multiple organ failure and mortality.Objectives:Tо analyze demographic, clinical and laboratory parameters, timing of MAS and disease outcome in patients (pts) with MAS and RD.Methods:The study included all pts of single center with RD, who developed the MAS. The diagnosis was recognized according to Classification criteria for MAS in sJIA [1].Results:We observed 52 pts with RD and MAS: 31 (59.6%) with sJIA, 19 (36.5%) – SLE, 1(1.9%) – juvenile dermatomyositis (JDM), 1 (1.9%) – overlap syndrome (JIA+JDM). Pts with MAS accounted of 26% of all pts with sJIA, 7.6% of all pts with SLE. The mean age of pts at onset of sJIA was 2.6 y [1.5; 5.75], at onset of SLE – 11.8 y [8.6; 13.95]. The patient with JDM was 6.5 years old, pts with overlap syndrome – 3.5 years old. Male to female ratio was 1:1,7. A total of 63 episodes of MAS was recorded (41 – in sJIA, 20 – SLE, 1 – JDM, 1 - overlap syndrome). 23 pts (44.2%) had MAS at onset, 26 pts (50%) – “classic” MAS (during the course of disease), 3 pts (5.8%) – recurrent MAS. The clinical manifestations of MAS included fever (90.4%), hepatomegaly (50%), pericarditis (46.2%), hemorrhagic rash (32.7%), neurologic involvement (42.3%), lung involvement (34.6%). We found hyperferritinemia in 98%, thrombocytopenia in 78.8%, increased transaminases in 88.5%, hypofibrinogenemia in 40.4%, hypertriglyceridemia in 42.3 % of pts. Most severe course of MAS was established in pts with ferritin levels >1500 ng/ml and with hypertriglyceridemia more than 6.0 mmol/l at an early stage. Bone marrow investigation was performed in 21 pts, and the evidence of haemophagocytosis was confirmed in 8 pts (38%). First features of MAS were fever, sleepiness, lower platelet counts, high TA. Lesions of the skin and mucous were mainly represented by point hemorrhages at an early stage in pts with SLE and MAS, the development of a bright rash with itching was typical for pts with sJIA and MAS. There are no principal differences between course of MAS in sJIA and other RD in children, but mild “subclinical” cases of MAS were observed only in pts with sJIA on biologics. For treatment of MAS all pts were administered high dose of glucocorticoids (per os+iv). Pts with SLE received: cyclophosphamide iv - 5 (26.3%), cyclosporine per os 1 (5.2%), IVIGs - 6 (31.6%), rituximab - 2 pts (10.5%). Pts with sJIA received: cyclosporine per os - 20 (64.5%), IVIGs - 25 (80.6%), 1 etoposide - (3.2%). Patient with overlap syndrome received cyclosporine per os. 8 pts (15.4%) died from MAS (3 with sJIA, 5 – with SLE).Conclusion:MAS can develop in various children’s RD, but more often in pts with sJIA. In our observation more pts had MAS during the course of disease, not at onset. Rapid recognition of MAS can improve treatment outcomes and prognosis.References:[1]Ravelli A, Minoia F, Davì S, et al. 2016.Disclosure of Interests:None declared

scholarly journals Macrophage Activation Syndrome in Adults: A Retrospective Case Series

2021 ◽  
Vol 9 ◽  
pp. 232470962110264
Author(s):  
Taylor Warmoth ◽  
Malvika Ramesh ◽  
Kenneth Iwuji ◽  
John S. Pixley
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Pediatric Patients ◽  
Inflammatory Diseases ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Case Series ◽  
Adult Patients ◽  
Retrospective Case ◽  
Life Threatening ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a form of hemophagocytic lymphohistocytosis that occurs in patients with a variety of inflammatory rheumatologic conditions. Traditionally, it is noted in pediatric patients with systemic juvenile idiopathic arthritis and systemic lupus erythematous. It is a rapidly progressive and life-threatening syndrome of excess immune activation with an estimated mortality rate of 40% in children. It has become clear recently that MAS occurs in adult patients with underlying rheumatic inflammatory diseases. In this article, we describe 6 adult patients with likely underlying MAS. This case series will outline factors related to diagnosis, pathophysiology, and review present therapeutic strategies.

scholarly journals High-dose anakinra as treatment for macrophage activation syndrome caused by refractory Kawasaki disease in an infant

2019 ◽  
Vol 12 (8) ◽  
pp. e229708 ◽  
Author(s):  
Marie Lind-Holst ◽  
Ulla Birgitte Hartling ◽  
Anne Estmann Christensen
Keyword(s):  
Pericardial Effusion ◽  
Kawasaki Disease ◽  
Coronary Arteries ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Additional Treatment ◽  
High Dose ◽  
Acute Phase Reactants ◽  
High Dose Methylprednisolone ◽  
Activation Syndrome

We report a 12-week-old boy presenting with incomplete refractory Kawasaki disease (KD) complicated with macrophage activation syndrome (MAS). The infant presented with cerebral irritability, pain, tachypnoea and vomiting for 10 days. He did not fulfil any of the classic diagnostic criteria for KD. Pericardial effusion on echocardiography in addition to severe dilatation of the coronary arteries in combination with leucocytosis and raised acute phase reactants led to the diagnosis of incomplete KD. Treatment with intravenous immunoglobulin and aspirin was initiated but without any response. The condition was subsequently refractory to additional treatment with infliximab and high-dose methylprednisolone. His condition worsened, fulfilling the criteria for MAS. High-dose anakinra was initiated, and remission of the inflammation was achieved.

scholarly journals Macrophage activation syndrome in MDA5 antibody-positive dermatomyositis and COVID-19 infection

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Marzieh Keshtkarjahromi ◽  
Sumit Chhetri ◽  
Amulya Balagani ◽  
Umm-ul-Banin B. Tayyab ◽  
Christopher J. Haas
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
High Dose ◽  
Potential Contribution ◽  
Clinical Reminder ◽  
High Dose Methylprednisolone ◽  
History Of ◽  
Rheumatological Disease ◽  
System Disorder ◽  
Activation Syndrome

Abstract Background Macrophage activation syndrome (MAS) is a rare multiorgan system disorder that may present as a fatal complication of underlying rheumatological disease, including dermatomyositis. Case presentation Here, we report the case of a 65-year-old Caucasian female with a history of psoriasis and a recent diagnosis of Coronavirus disease 2019 (COVID-19) who presented with progressive generalized weakness, joint pains, an erythematous rash, shortness of breath, and weight loss. She was ultimately diagnosed with biopsy-confirmed melanoma differentiation-associated protein 5 (MDA5)-positive dermatomyositis complicated by MAS, requiring intravenous immunoglobulin and high-dose methylprednisolone. Conclusions This report serves as a clinical reminder of the rare, yet clinically relevant association between MDA5-positive dermatomyositis and MAS, as well as highlights the potential contribution of other immune system activating diseases, such as COVID-19, associated with a cytokine storm and hyperinflammatory state.

scholarly journals P022 Anakinra for use in non- juvenile idiopathic arthritis (JIA) related haemophagocytic lymphohistiocytosis (HLH): evidence base and funding

2019 ◽  
Vol 104 (7) ◽  
pp. e2.26-e2
Author(s):  
Octavio Aragon Cuevas
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Case Reports ◽  
Case Series ◽  
Evidence Base ◽  
British Society ◽  
Paediatric Patients ◽  
Activation Syndrome

BackgroundNon JIA related HLH is a life-threatening complication that is increasingly recognised in paediatric patients, particularly in those who are unwell in the paediatric intensive care unit (PICU). Untreated or insufficiently treated HLH has a significant mortality rate (up to 53%).1AimTo review the evidence base for the use of anakinra in paediatric patients with non-JIA HLH refractory to systemic corticosteroids in patients who are not fit for treatment as per HLH 2004 protocol.MethodsA PubMed search with words ‘anakinra’ and ‘hemophagocytic lymphohistiocytosis’ was carried out on July 2018 to find out the evidence base with regards to the use of anakinra in non-JIA related HLH. Any published peer reviewed clinical studies or trials (including but not limited to retrospective or prospective controlled trials, comparative studies and observational/cohort studies) were considered. Case reports and series were considered if better evidence studies were not available. A recent case study from a tertiary paediatric centre will be used to illustrate the pathway followed to diagnose non-JIA related HLH and funding options.ResultsAlthough a protocol exists for primary HLH treatment (HLH 2004), including chemotherapy and stem cell transplantation,2 there is no consensus on how to treat secondary HLH. The literature mainly showed case reports and small case series,3 describing the use of anakinra collectively for 35 patients (median age 14 to 48 years) who met the HLH 2004 diagnostic criteria with an overall survival rate of up to 88% at time of discharge from the PICU3. Anakinra was used at standard doses always in combination with corticosteroids. Some patients also received intravenous immunoglobulin (IVIG) and ciclosporin at the discretion of the medical teams.ConclusionThe evidence for use of anakinra in non JIA secondary HLH is limited to retrospective observational studies and mostly restricted to adult populations. Despite this caveat, these studies have demonstrated that anakinra therapy alongside other non-etoposide immunomodulatory therapies is associated with an improvement in short term survival. In patients with multi-organ dysfunction, who are too unstable to receive the existing etoposide based HLH-2004 treatment regimen due to concerns regarding significant treatment toxicity, personalised non-etoposide therapies including dexamethasone, IVIG, ciclosporin and anakinra may be better tolerated and provide a bridge to future more standardised treatment. Evidence to date shows that relapse of secondary HLH is possible with ciclosporin therapy. In JIA related HLH, anakinra was considered better than ciclosporin at inducing remission and having a lower incidence of adverse effects,4 and NHS England granted funding for the treatment based on these findings. The available evidence did not show any serious adverse events related to anakinra.RecommendationsThis tertiary centre approved the use of anakinra for this patient and future patients with this indication despite lack of reimbursement from NHS England for the drug. An urgent interim policy review will be put together by a team of the British Society of Paediatric and Adolescent Rheumatology (BSPAR) and presented to the NHS England commissioners to seek funding for anakinra for paediatric patients with this indicationReferencesMiettunen, 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 (Oxford) 2011;50:417–9Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124–31.Kumar B, Aleem S, Saleh H, Petts J, Ballas ZK. A Personalized Diagnostic and Treatment Approach for Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Adults. J Clin Immunol 2017;37:638–643Boom V, et al. Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Pediatr Rheumatol Online J 2015;13:55.

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