scholarly journals Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects

2021 ◽  
Vol 8 ◽  
Author(s):  
Altynay Abdirakhmanova ◽  
Vitaliy Sazonov ◽  
Zaure Mukusheva ◽  
Maykesh Assylbekova ◽  
Diyora Abdukhakimova ◽  
...  
Keyword(s):  
Systematic Review ◽  
Systemic Lupus Erythematosus ◽  
Diagnostic Criteria ◽  
Rheumatic Diseases ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus ◽  
Activation Syndrome

Macrophage Activation Syndrome (MAS) is a very severe complication of different rheumatic diseases, including pediatric Systemic Lupus Erythematosus (pSLE). MAS is not considered as a frequent complication of pSLE; however, its occurrence could be under-estimated and the diagnosis can be challenging. In order to address this issue, we performed a systematic review of the available medical literature, aiming to retrieve all those papers providing diagnostic (clinical/laboratory) data on patients with pSLE-related MAS, in individual or aggregated form. The selected case reports and series provided a pool of 46 patients, accounting for 48 episodes of MAS in total. We re-analyzed these patients in light of the diagnostic criteria for MAS validated in systemic Juvenile Idiopathic Arthritis (sJIA) patients and the preliminary diagnostic criteria for MAS in pSLE, respectively. Five clinical studies were also selected and used to support this analysis. This systematic review confirms that MAS diagnosis in pSLE patients is characterized by several diagnostic challenges, which could lead to delayed diagnosis and/or under-estimation of this complication. Specific criteria should be considered to diagnose MAS in different rheumatic diseases; as regards pSLE, the aforementioned preliminary criteria for MAS in pSLE seem to perform better than the sJIA-related MAS criteria, because of a lower ferritin cut-off.

scholarly journals A15: Predicting Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus Patients at Diagnosis

2014 ◽  
Vol 66 ◽  
pp. S25-S25 ◽  
Author(s):  
Maya Gerstein ◽  
Sharon Sukhdeo ◽  
Deborah M. Levy ◽  
Brian M. Feldman ◽  
Susanne M. Benseler ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus ◽  
Activation Syndrome

P043 Predictors of mortality in childhood-onset Systemic Lupus Erythematosus

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
O Gacem ◽  
Z Zeroual ◽  
Z Arrada ◽  
Moussa Achir ◽  
M S Ladj
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Interquartile Range ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus ◽  
The Mean ◽  
Activation Syndrome

Abstract Background Pediatric Systemic Lupus Erythematosus (pSLE) is a severe autoimmune disease due to its serious multi-visceral disorders, its morbidity causing deleterious and sometimes permanent effects. The objective is to determine the prognostic predictive factors of death in pSLE. Methods This was a prospective, descriptive, multicentre study, including patients less than16 years of age with SLE according to the criteria SLE of the American College of Rheumatology over a period of 36 months (2015–2018) carried out in the CHU Nefissa Hamoud in Algiers. The number of deaths during this follow-up period was determined and compared with surviving patients in several parameters (demographic, clinical, laboratory, therapeutic and disease activity). Disease activity was estimated by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), the use of which has been validated in children and damage and sequelae were appreciated by the Systemic Lupus International Collaborating Clinics (SLICC). Means, percentages and χ2 tests were specified. The multivariate analysis of logistic regression was used to determine prognostic factors associated with the outcome variable. P values <0.05 were considered statistically significant. Results 83 patients were collected and divided into two groups: deceased and survivors in which different parameters were compared and analyzed. Results: Female: male ratio 1: 4.9, mean age at diagnosis 11.3 ± 3.62. Clinical involvements at diagnosis were: cutaneous (100%), hematological (79.5%), articular (65, 1%), renal (44, 6%), neurological (41%), hepato-digestive (41%), cardiac (27, 7%), pleuropulmonary (19, 3%) and ocular involvement (7, 2%). The mean of SLEDAI was 22.11 ± 11.87 [interquartile range 2–53]. The prevalence of death was 11% of cases with an average age of death of 11 years [interquartile range 5–15 years]. The comparison of the two groups demonstrated a significant association between death and the following parameters: renal and neurological damage respectively: ((P = 0.03), (P = 0.02)), macrophage activation syndrome (MAS) (P = 0.0002), infections (P = 0.027), disease activity (P = 0.006) and damage score (P = 0.001). The mean SLEDAI activity in deceased patients was 32 ± 11.9 [interquartile range 17–53], compared with surviving patients which was 20.9 ± 11.4 [interquartile range 2–51]. Multivariate analysis with logistic regression revealed two major predictors of death, namely neurological involvement (odds ratio = 6,093 95% confidence interval ((1,1 8 0 ∼ 31 446)) and macrophage activation syndrome. Adherence to treatment was a protective factor: ([0.016 (0.001–0.353)]. Conclusion These results showed that pediatric systemic lupus erythematosus exhibits an aggressive and severe phenotype with an unpredictable course. Studies are needed in children in order to specify and develop prognostic predictive factors and to identify patients at high risk of early mortality in order to design early and effective care of this vulnerable entity of lupus disease. Keywords Child, Systemic Lupus Erythematosus, mortality

scholarly journals Hyperferritinemia Wins Again: Defining Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus

2021 ◽  
pp. jrheum.210024
Author(s):  
Emily A. Smitherman ◽  
Randy Q. Cron
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus ◽  
Threatening Condition ◽  
Life Threatening ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a potentially life-threatening condition of hyperinflammation that can be secondary to an underlying chronic rheumatic condition, commonly systemic juvenile idiopathic arthritis (sJIA) but also childhood-onset systemic lupus erythematosus (cSLE). MAS is characterized by excessive activation of T lymphocytes and macrophages that lead to overproduction of cytokines and results in cytopenia, liver dysfunction, and coagulopathy1.

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.

scholarly journals Macrophage Activation Syndrome, Glomerulonephritis, Pericarditis, and Retinal Vasculitis as Initial Presentation of Systemic Lupus Erythematosus

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Yiming Luo ◽  
Yumeng Wen ◽  
Ana Belen Arevalo Molina ◽  
Punya Dahal ◽  
Lorenz Leuprecht ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Retinal Vasculitis ◽  
Initial Presentation ◽  
Systemic Lupus ◽  
First Case ◽  
Multisystem Involvement ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a rare manifestation of systemic lupus erythematosus (SLE) with potentially life-threatening consequences. To the best of our knowledge, this is the first case reported in literature for a constellation of MAS, glomerulonephritis, pericarditis, and retinal vasculitis as initial presentation of SLE. Despite extensive multisystem involvement of his disease, the patient responded well to initial steroid treatment, with mycophenolate mofetil successfully added as a steroid-sparing agent. Our case highlights the importance of multispecialty collaboration in the diagnosis and management of SLE with multisystem involvement.

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