scholarly journals Intravenous administration of anakinra in children with macrophage activation syndrome

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Omkar Phadke ◽  
Kelly Rouster-Stevens ◽  
Helen Giannopoulos ◽  
Shanmuganathan Chandrakasan ◽  
Sampath Prahalad
Keyword(s):  
Intravenous Administration ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Therapeutic Option ◽  
Interleukin 1 ◽  
Neurological Dysfunction ◽  
Systemic Jia ◽  
Subcutaneous Edema ◽  
Activation Syndrome

Abstract Background Subcutaneous anakinra is an interleukin-1 inhibitor used to treat juvenile idiopathic arthritis. Recent reports suggest anakinra can be a valuable addition to the treatment of COVID-19 associated cytokine storm syndrome and the related multisystem inflammatory syndrome (MIS-C) in children. Herein, we describe our experience with intravenously administered anakinra. Findings 19 Patients (9 male) received intravenous (IV) anakinra for treatment of macrophage activation syndrome (MAS) secondary to systemic lupus erythematosus (SLE), systemic JIA (SJIA) or secondary hemophagocytic lymphohistiocytosis (sHLH). In most cases the general trend of the fibrinogen, ferritin, AST, and platelet count (Ravelli criteria) improved after initiation of IV anakinra. There were no reports of anaphylaxis or reactions associated with administration of IV anakinra. Conclusion Intravenous administration of anakinra is an important therapeutic option for critically ill patients with MAS/HLH. It is also beneficial for those with thrombocytopenia, subcutaneous edema, neurological dysfunction, or very young, hospitalized patients who need multiple painful subcutaneous injections.

Association ofIRF5Polymorphisms with Susceptibility to Macrophage Activation Syndrome in Patients with Juvenile Idiopathic Arthritis

2011 ◽  
Vol 38 (4) ◽  
pp. 769-774 ◽  
Author(s):  
MASAKATSU YANAGIMACHI ◽  
TAKUYA NARUTO ◽  
TAKAKO MIYAMAE ◽  
TAKUMA HARA ◽  
MASAKO KIKUCHI ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Proinflammatory Cytokines ◽  
Lupus Erythematosus ◽  
Gene Polymorphisms ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Nucleotide Polymorphisms ◽  
Systemic Jia ◽  
Increased Risk ◽  
Activation Syndrome

Objective.Systemic-onset juvenile idiopathic arthritis (systemic JIA) and macrophage activation syndrome (MAS), the most devastating complication of systemic JIA, are characterized by abnormal levels of proinflammatory cytokines. Interferon regulatory factor 5 (IRF5) is a member of the IRF family of transcription factors, and acts as a master transcription factor in the activation of genes encoding proinflammatory cytokines. Polymorphisms in theIRF5gene have been associated with susceptibility to autoimmune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis. Our aim was to assess associations ofIRF5gene polymorphisms with susceptibility to systemic JIA and MAS.Methods.ThreeIRF5single-nucleotide polymorphisms (rs729302, rs2004640, and rs2280714) were genotyped using TaqMan assays in 81 patients with systemic JIA (33 with MAS, 48 without) and 190 controls.Results.There were no associations of theIRF5gene polymorphisms or haplotypes under study with susceptibility to systemic JIA. There was a significant association of the rs2004640 T allele with MAS susceptibility (OR 4.11; 95% CI 1.84, 9.16; p = 0.001). TheIRF5haplotype (rs729302 A, rs2004640 T, and rs2280714 T), which was reported as conferring an increased risk of SLE, was significantly associated with MAS susceptibility in patients with systemic JIA (OR 4.61; 95% CI 1.73, 12.3; p < 0.001).Conclusion.IRF5gene polymorphism is a genetic factor influencing susceptibility to MAS in patients with systemic JIA, and IRF5 contributes to the pathogenesis of MAS in these patients.

scholarly journals Visceral Leishmaniasis Associated with Macrophage Activation Syndrome and Diffuse Alveolar Hemorrhage in a Lupus Patient

2018 ◽  
Vol 31 (10) ◽  
pp. 593
Author(s):  
Andreia Costa ◽  
Cármen Pais ◽  
Sofia Cerqueira ◽  
Fernando Salvador
Keyword(s):  
Amphotericin B ◽  
Lupus Erythematosus ◽  
Liposomal Amphotericin ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Liposomal Amphotericin B ◽  
Leishmania Parasites ◽  
Activation Syndrome

Systemic lupus erythematosus is a heterogeneous and unpredictable autoimmune disease which can be complicated to approach and treat. Hemophagocytic lymphohistiocytosis and diffuse alveolar hemorrhage are rare disease complications. The authors describe a clinical case of a 32-year-old woman with lupus and fever of unknown origin. From the investigations performed, the myelogram revealed hemophagocytosis and Leishmania parasites, therefore liposomal amphotericin B was then started. In addition to directed therapy, she maintained fever that evolved with diffuse alveolar hemorrhage. The myelogram was repeated and showed that she still had hemophagocytosis but now without parasites. Corticotherapy was increased and intravenous Immunoglobulin was started, with improvement. Rituximab was started as a result of macrophage activation syndrome and diffuse alveolar hemorrhage. Months after discharge, she began once again to have sustained fever and Leishmania parasites were found again, therefore liposomal amphotericin B was started once more associated with miltefosine. She continues being followed-up as she is asymptomatic and using steroidsin weaning scheme.

scholarly journals Arthritis and use of hydroxychloroquine associated with a decreased risk of macrophage activation syndrome among adult patients hospitalized with systemic lupus erythematosus

Lupus ◽  
2018 ◽  
Vol 27 (7) ◽  
pp. 1065-1071 ◽  
Author(s):  
E M Cohen ◽  
K D’Silva ◽  
D Kreps ◽  
M B Son ◽  
K H Costenbader
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Hospital Admission ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Activity Index ◽  
Conditional Logistic Regression ◽  
Systemic Lupus ◽  
Factors Associated ◽  
Activation Syndrome

Background Macrophage activation syndrome (MAS) is an uncommon but serious complication of systemic lupus erythematosus (SLE). We aimed to identify factors associated with MAS among adult hospitalized SLE patients. Methods Within the Brigham and Women’s Hospital (BWH) Lupus Center Registry, we identified adult SLE patients > age 17 who had been hospitalized from 1970 to 2016, with either ferritin > 5000 ng/ml during admission or “macrophage activation syndrome” or “MAS” in discharge summary. We confirmed MAS by physician diagnosis in medical record review. We matched each hospitalized SLE patient with MAS to four SLE patients hospitalized without MAS (by SLE diagnosis date ±1 year). We employed conditional logistic regression models to identify clinical factors associated with MAS among hospitalized SLE patients. Results Among 2094 patients with confirmed SLE, we identified 23 who had a hospitalization with MAS and compared them to 92 hospitalized without MAS. Cases and controls had similar age at SLE diagnosis (29.0 vs. 30.5, p = 0.60), and hospital admission (43.0 vs. 38.3, p = 0.80), proportion female (78% vs. 84%, p = 0.55), and time between SLE diagnosis and hospitalization (1971 vs. 1732 days, p = 0.84). Arthritis (OR 0.04 (95% CI 0.004–0.35)) and hydroxychloroquine use (OR 0.18 (95% CI 0.04–0.72)) on admission were associated with decreased MAS risk. Admission Systemic Lupus Erythematosus Disease Activity Index scores (30 vs. 19, p = 0.002) and lengths of stay (16 days vs. 3 days, p < 0.0001) were much higher among cases. Death during hospitalization was 19% among cases and 3% among controls ( p = 0.03). Conclusions In this case-control study of hospitalized adult SLE patients, arthritis and hydroxychloroquine use at hospital admission were associated with decreased MAS risk. Further studies are needed to validate these factors associated with lowered MAS risk.

P034 Macrophage activation syndrome in Juvenile Idiopathic Arthritis

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Rahma Guedri ◽  
Glaimeriem ◽  
Zohra Fitouri ◽  
Saayda Ben Becher ◽  
Bechir Hamza
Keyword(s):  
Biological Treatment ◽  
Pediatric Rheumatology ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Jia ◽  
Rheumatology Unit ◽  
The Mean ◽  
And Biological Markers ◽  
Activation Syndrome

Abstract Objectives To describe the epidemiological, clinical-biological, and therapeutic characteristics of children with systemic JIA complicated with a macrophage activation syndrome (MAS). Methods It was a retrospective study, including children with JIA followed at the pediatric rheumatology unit of the children's hospital in Tunis for 22 years (January 1999 to December 2020), and presented MAS during their follow-up. Results We included 40 patients with JIA. Nineteen children (47.5%) presented MAS during the disease. They are 11 boys and 8 girls, with a sex ratio of 1.3. The mean age was 5.31 years (range: 0.66–10.83 years). The circumstances of the occurrence were variable. MAS was inaugural in 10 patients. Three of our patients presented MAS twice. MAS was definitive with clinical and biological markers in 12 cases and only biological in 7 cases. Seventeen of our patients were treated with intravenous Corticosteroids. Seven Childs among them received, in combination, Immunoglobulins (IG). We prescribed a biological treatment in 3 patients. We have mourned only one death. Conclusion MAS is a severe complication of JIA and is the leading cause of death.

Sign in / Sign up

Export Citation Format

Share Document