scholarly journals Macrophage activation syndrome as a complication of rheumatologic disorders, a report from Iran

Reumatismo ◽  
2020 ◽  
Vol 71 (4) ◽  
pp. 189-198 ◽  
Author(s):  
R. Assari ◽  
P. Sadeghi ◽  
A. Mirmohammadsadeghi ◽  
F. Ebadi ◽  
V. Ziaee
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Platelet Count ◽  
Lupus Erythematosus ◽  
Laboratory Data ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Underlying Disease ◽  
Disease Group ◽  
Rheumatologic Disease ◽  
Recurrent Group ◽  
Presentation Group

The objective was to evaluate the clinical and laboratory manifestations and outcomes of the MAS cases in the context of systemic juvenile idiopathic arthritis (SJIA), systemic lupus erythematosus (SLE), Kawasaki disease, poly-articular juvenile idiopathic arthritis (PJIA). Twenty consecutive patients diagnosed with MAS between 2005 and 2016 entered the study. The cases were divided into two groups: in the first, MAS emerged in the context of a previously diagnosed rheumatologic disease, while in the second, MAS was the first presentation of a rheumatologic disease. In the other classification, the cases were divided into recurrent and non-recurrent cases. Laboratory data were recorded at three times: before MAS attack, during MAS attack, and 1 month after discharge from hospital. Nineteen cases with the median age of 5.9 (3.6-10) years entered the study. Four cases (21.1%) showed recurrent attacks of MAS. MAS was the first presentation of disease in 10 cases. The median age of the patients in the underlying disease group (10 years) was significantly higher than in the first presentation group [4.5(1.7-6.1) years, p=0.003]. The median fibrinogen value during MAS attack in the underlying disease group (601 mg/ dL) was also significantly higher than in the first presentation group (174 mg/dL, p=0.038). The platelet count during MAS attack in the recurrent group (30,500/microliter) was significantly lower than in the non-recurrent group (135,000/microliter, p=0.042). Our series of MAS cases demonstrated an overview of the symptoms, signs, laboratory manifestations, treatment, and prognosis of these cases. The higher median fibrinogen in MAS in the underlying disease group revealed that a decreasing level of fibrinogen in chronic disease is more significant than a single cut off value. Indeed, the lower platelet count in the recurrent MAS group may indicate greater platelet consumption due to organomegaly. Early diagnosis and treatment may save the patients’ lives.

scholarly journals A single indicator joint in non-systemic juvenile idiopathic arthritis whose ultrasound scores are correlated with disease activity

2020 ◽  
Author(s):  
Yung-Hsien Huang ◽  
Ya-Chiao Hu ◽  
Chun-Hua Liao ◽  
Bor-Luen Chiang ◽  
Cheng‐Hsun Lu ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Disease Activity ◽  
Power Doppler ◽  
Laboratory Data ◽  
Health Assessment ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Disease Classification ◽  
Physician Global Assessment ◽  
Single Indicator ◽  
Systemic Jia

Abstract Background: Musculoskeletal ultrasound (MSUS) has been used worldwide in adult patients with rheumatoid arthritis (RA) but not in juvenile idiopathic arthritis (JIA). The aim of this study was to investigate the application of MSUS findings of a single indicator joint in JIA to assess the disease activity and classify disease subtype.Methods: Thirty-five non-systemic JIA patients with a total of 62 visits were retrospectively recruited in this study. Among involved joints, the one with highest value of grey scale (GS) plus power Doppler (PD) (=GSPD) was selected as the indicator joint of each visit. The correlations between MSUS parameters of indicator joints and the Physician Global Assessment (PGA) score, The Childhood Health Assessment Questionnaire‐disability index (CHAQ-DI), and laboratory data were analyzed. The ultrasound features in different subtypes of JIA were also compared.Results: PD was weakly correlated with the PGA score (rho=0.323, p=0.010), while both GS and GSPD were moderately correlated with the PGA score (rho=0.405, p=0.001; rho=0.434, p=0.000). On the other hand, GS, PD, and GSPD were weakly correlated with CHAQ-DI. Although erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) had a weak correlation with PGA, they were not statistically correlated with GS, PD, or GSPD. The proportions of effusion, synovial hypertrophy, and enthesopathy in 3 different subtypes, showed significant differences (Fisher’s exact test, p=0.037; p=0.004; p=0.019). Enthesopathy was only seen in joints of enthesitis-related arthritis (ERA) but not in joints of polyarthritis and oligoarthritis.Conclusions: MSUS seems to be an acceptable non-invasive tool for JIA, particularly for non-systemic JIA patients, that could assist disease classification, and whose parameters of the indicator joints may potentially contribute to the disease activity evaluation.

scholarly journals Utilizing ultrasound findings of a single indicator joint to assess non-systemic juvenile idiopathic arthritis

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Yung-Hsien Huang ◽  
Ya-Chiao Hu ◽  
Chun-Hua Liao ◽  
Bor-Luen Chiang ◽  
Cheng-Hsun Lu ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Disease Activity ◽  
Power Doppler ◽  
Laboratory Data ◽  
Health Assessment ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Disease Classification ◽  
Physician Global Assessment ◽  
Single Indicator ◽  
Systemic Jia

Abstract Background Musculoskeletal ultrasound (MSUS) has been used worldwide in adult patients with rheumatoid arthritis (RA) but is beginning to play an increasing role in patients with juvenile idiopathic arthritis (JIA). The aim of this study was to investigate the application of MSUS findings of a single indicator joint in JIA to assess the disease activity and classify disease subtype. Methods Thirty-five non-systemic JIA patients with a total of 62 visits were retrospectively recruited in this study. Among the involved joints, the joint with highest value of grey-scale (GS) plus power Doppler (PD) (=GSPD) was selected as the indicator joint at each visit. The correlations between each MSUS parameter (GS, PD, GSPD) of indicator joints and the Physician Global Assessment (PGA) score, the Childhood Health Assessment Questionnaire-disability index (CHAQ-DI), and laboratory data were analyzed. The ultrasound features in different subtypes of JIA were also compared. Results PD was weakly correlated with the PGA score (rho = 0.323, p = 0.010), while both GS and GSPD were moderately correlated with the PGA score (rho = 0.405, p = 0.001; rho = 0.434, p = 0.000). On the other hand, GS, PD, and GSPD were weakly correlated with CHAQ-DI. Although erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) had a weak correlation with PGA, they were not statistically correlated with GS, PD, or GSPD. The proportions of effusion, synovial hypertrophy, and enthesopathy in three different subtypes, showed significant differences (Fisher’s exact test, p = 0.037; p = 0.004; p = 0.019). Enthesopathy was only seen in joints of enthesitis-related arthritis (ERA), but not in joints of polyarthritis and oligoarthritis. Conclusions MSUS is an acceptable non-invasive tool for the patients with JIA, particularly for those with non-systemic JIA, that might assist disease classification, and whose parameters of the indicator joints may potentially contribute to the evaluation of disease activity.

scholarly journals Risk Factors of Persistently Active Disease among Filipino Children with Systemic Juvenile Idiopathic Arthritis: a 10-Year Study in a Tertiary Hospital

2021 ◽  
Vol 5 (1) ◽  
pp. 621-627
Author(s):  
Ivy Joy E Alberca ◽  
MA. Theresa M Collante ◽  
Christine B Bernal
Keyword(s):  
Risk Factors ◽  
Juvenile Idiopathic Arthritis ◽  
Platelet Count ◽  
Disease Activity ◽  
Functional Limitations ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Tertiary Hospital ◽  
Joint Involvement ◽  
Filipino Children ◽  
Active Disease

Background: Systemic juvenile idiopathic arthritis (SJIA) is one of the most common subtypes of arthritis among children in southeast Asia with higher progression of disease activity. Unsuccessful control of the disease may lead to long-term disability resulting in functional limitations that would affect productivity of the individual. Objective: The study determined the risk factors for persistently active disease among Filipino children aged 2 weeks to 18 years diagnosed with SJIA seen in the Section of Pediatric Rheumatology of the University of Santo Tomas Hospital (USTH) from June 2009 to June 2019. Methodology: A retrospective cohort study was done involving chart review of both clinical division and private division patients. The following parameters were determined: sex, age at diagnosis, time elapsed from symptom onset to diagnosis, joint involvement, inflammatory markers and extra-articular manifestation. Statistical analysis included frequencies, percentages and logistic regression for the risk factors of interest. Results: One hundred twenty-seven patients with SJIA who were appropriately treated for at least three years were included. Among which, 88 (69%) developed persistently active disease. Among them, 36 (41%) were diagnosed at 1-5 years old. Many were diagnosed (n=54, 61%) after five weeks. The most commonly affected joints were the wrists, knees and ankles. The most common contracture noted involved the cervical joint. Only 33 (26%) patients received biologic agents. Risk factors identified for the development of persistent disease activity were low hemoglobin levels at the time of diagnosis and after one month of treatment, elevated platelet count after a month, substantial joint count after three months and increased ESR after 6 months. Conclusion: The change or improvement of the joint count and in hemoglobin, platelet count and ESR levels after appropriate treatment may determine the risk for persistently active disease in Filipino children with SJIA.

Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis under Treatment with Tocilizumab

2015 ◽  
Vol 42 (4) ◽  
pp. 712-722 ◽  
Author(s):  
Shumpei Yokota ◽  
Yasuhiko Itoh ◽  
Tomohiro Morio ◽  
Naokata Sumitomo ◽  
Kaori Daimaru ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Clinical Laboratory ◽  
Laboratory Data ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Clinical Manifestations ◽  
Safety Evaluation ◽  
Laboratory Findings ◽  
Activation Syndrome

Objective.To identify macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA) undergoing tocilizumab (TCZ) treatment, and to confirm laboratory marker changes and responses to treatment in patients with MAS receiving TCZ.Methods.In Japan, 394 patients with sJIA were registered in an all-patient registry surveillance of TCZ as of January 15, 2012. TCZ (8 mg/kg) was administered every 2 weeks to patients with sJIA. MAS, hemophagocytic lymphohistiocytosis, or Epstein-Barr virus–associated hemophagocytic syndrome (EB-VAHS) was reported in 23 of these patients (25 events). The Safety Evaluation Committee of Tocilizumab for JIA reviewed these cases and clinically evaluated the data and laboratory findings using their own therapeutic experience. Events were categorized into 4 groups: definitive MAS, probable MAS, EB-VAHS, and non-MAS.Results.The committee’s review revealed 3 events of definitive MAS in 3 patients, 12 events of probable MAS in 11 patients, 2 events of EB-VAHS in 2 patients, and 8 events of non-MAS in 8 patients. There were 2 patients who developed 2 events: 2 events in 1 patient were classified into definitive MAS and probable MAS, and 2 events in another patient were classified into probable MAS. In patients with definitive or probable MAS, common clinical manifestations and laboratory findings of MAS were observed. Changes in laboratory data observed in patients with EB-VAHS were similar to those observed in patients with MAS.Conclusion.These results suggest that the clinical/laboratory features in the course of MAS appear to be similar among patients regardless of whether TCZ is administered. Similarities in the pathophysiological background of MAS and EB-VAHS were also suggested.

scholarly journals Utilizing ultrasound findings of a single indicator joint to assess non-systemic juvenile idiopathic arthritis

2020 ◽  
Author(s):  
Yung-Hsien Huang ◽  
Ya-Chiao Hu ◽  
Chun-Hua Liao ◽  
Bor-Luen Chiang ◽  
Cheng‐Hsun Lu ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Disease Activity ◽  
Power Doppler ◽  
Laboratory Data ◽  
Health Assessment ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Disease Classification ◽  
Physician Global Assessment ◽  
Single Indicator ◽  
Systemic Jia

Abstract Background: Musculoskeletal ultrasound (MSUS) has been used worldwide in adult patients with rheumatoid arthritis (RA) but is beginning to play an increasing role in patients with juvenile idiopathic arthritis (JIA). The aim of this study was to investigate the application of MSUS findings of a single indicator joint in JIA to assess the disease activity and classify disease subtype.Methods: Thirty-five non-systemic JIA patients with a total of 62 visits were retrospectively recruited in this study. Among the involved joints, the joint with highest value of grey-scale (GS) plus power Doppler (PD) (=GSPD) was selected as the indicator joint at each visit. The correlations between each MSUS parameter (GS, PD, GSPD) of indicator joints and the Physician Global Assessment (PGA) score, the Childhood Health Assessment Questionnaire‐disability index (CHAQ-DI), and laboratory data were analyzed. The ultrasound features in different subtypes of JIA were also compared.Results: PD was weakly correlated with the PGA score (rho=0.323, p=0.010), while both GS and GSPD were moderately correlated with the PGA score (rho=0.405, p=0.001; rho=0.434, p=0.000). On the other hand, GS, PD, and GSPD were weakly correlated with CHAQ-DI. Although erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) had a weak correlation with PGA, they were not statistically correlated with GS, PD, or GSPD. The proportions of effusion, synovial hypertrophy, and enthesopathy in three different subtypes, showed significant differences (Fisher’s exact test, p=0.037; p=0.004; p=0.019). Enthesopathy was only seen in joints of enthesitis-related arthritis (ERA), but not in joints of polyarthritis and oligoarthritis.Conclusions: MSUS is an acceptable non-invasive tool for the patients with JIA, particularly for those with non-systemic JIA, that might assist disease classification, and whose parameters of the indicator joints may potentially contribute to the evaluation of disease activity.

Changes of IL-4 and IFN-g expression in monocytes and T-helper lymphocytes while modeling of systemic juvenile idiopathic arthritis in Wistar rats

2018 ◽  
pp. 61-69
Author(s):  
В.Н. Сахаров ◽  
П.Ф. Литвицкий ◽  
Е.И. Алексеева ◽  
Н.А. Маянский ◽  
Р.Ш. Закиров
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Peripheral Blood Mononuclear Cells ◽  
Wistar Rats ◽  
Mononuclear Cells ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Control Group ◽  
Peripheral Blood Mononuclear ◽  
Treatment Groups ◽  
Blood Mononuclear Cells ◽  
T Helpers

Цель исследования - изучение перепрограммирования мононуклеарных лейкоцитов на модели системного ювенильного идиопатического артрита (сЮИА), воспроизводимой у крыс Wistar с использованием полного адъюванта Фрейнда и липополисахарида. Методика. сЮИА воспроизведен у 6-месячных крыс-самцов Wistar. На 40-е сут. эксперимента животные были разделены на 3 группы: 1-я группа - контроль; 2-я - группа доксициклина; 3-я - группа дексаметазона. Взятие проб крови у животных проводили на нулевые, 41-е и 55-е сут. Мононуклеарные клетки периферической крови выделяли гравиметрически, после чего окрашивали их на маркеры и внутриклеточные цитокины. Дифференцировали моноциты (CD3-CD4+) и Т-хелперы (CD3+CD4+). Анализировали динамику внутриклеточной экспрессии интерлейкина IL-4 (рассматривали как маркер про-М2 фенотипа, так как в случае выделения из клетки ИЛ-4 служит стимулятором М2 поляризации макрофагов) и IFN-g (как маркер про-М1 фенотипа) по данным проточной цитофлуориметрии. Применяли непараметрический статистический тест Mann-Whitney-Wilcoxon в программе R для статистической обработки данных. Результаты и заключение. При моделировании сЮИА выявлено закономерное изменение фенотипа моноцитов. Применение же доксициклина и дексаметазона приводило к более ранней поляризации их по про-М2-пути в отношении моноцитов (на 41-е сут.) в сравнении с контролем. Про-М1 эффект (на 55-е сут., в сравнении с контролем) выявлен также в группах доксициклина и дексаметазона. У животных разных групп обнаружены характерные динамические изменения внутриклеточной экспрессии цитокинов. Важно, что различная направленность поляризации фенотипа при сЮИА и применении препаратов наблюдается не только у моноцитов, но и у Т-хелперов. The study objective was to evaluate targeted reprogramming of peripheral blood mononuclear cells in systemic juvenile idiopathic arthritis (sJIA) modeled in 6-month-old male Wistar rats by co-administration of complete Freund’s adjuvant and lipopolysaccharide. Methods. On day 40 of the experiment, rats were divided into three groups: control, doxycycline, and dexamethasone groups. Blood samples were collected on days 0, 41, and 55. Peripheral blood mononuclear cells were isolated gravimetrically and stained for markers and cytokines. Monocytes (CD3-CD4+) and T-helpers (CD3+CD4+) were differentiated as target cells. IL-4 was considered a marker for the pro-M2 phenotype since IL-4 can activate M2 macrophage polarization; IFN-g was considered a marker for the pro-M1 phenotype. Time-related changes in the intracellular expression of IL-4 and IFN-g were studied using flow cytometry. Data were analyzed using nonparametric statistical tests (Mann-Whitney-Wilcoxon) in the R environment for statistical computing. Results and conclusions. Monocytes (like macrophages) underwent reprogramming during the development of modeled sJIA disease. In monocytes of doxycycline and dexamethasone treatment groups, pro-M2 effects were observed earlier (day 41) than in the control group. Pro-M1 effects were observed in monocytes of doxycycline and dexamethasone groups on day 55, as compared with the control group. Characteristic time-related changes of intracellular cytokine expression were described for different groups. Importantly, the differently directed phenotype polarization was observed in sJIA and treatment groups for both monocytes and T-helpers.

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