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Systemic juvenile idiopathic arthritis is one of the common rheumatic and immune diseases in children. It has a sudden onset, obvious systemic symptoms, and lung involvement. However, systemic juvenile idiopathic arthritis with an early manifestation of pulmonary ground-glass opacities combined with macrophage activation syndrome is rare. The clinical data of a child with systemic juvenile idiopathic arthritis with pulmonary ground-glass shadow and macrophage activation syndrome who was admitted to Hubei Maternal and Child Health Care Hospital affiliated to Tongji Medical College of Huazhong University of Science and Technology in December 2021 were analyzed retrospectively in order to improve the understanding of rheumatic diseases and pulmonary lesions. The child was admitted to the hospital for 10 days due to rash and fever. Thoracic CT showed scattered ground glass like shadows in both lungs due to the prevention and control screening of COVID-19 pneumonia epidemic situation. After admission, the child was still repeatedly flaccid with high fever, accompanied by dysfunction of both lower limbs. The knee joint MRI found that there was synovitis in the knee joint, and various laboratory indicators suggested macrophage activation syndrome. After that, systemic juvenile idiopathic arthritis was diagnosed. After being treated with methylprednisolone, cyclosporine and topzumab, the clinical remission and the ground-glass shadow of the lung basically disappeared. Through the analysis of this case, it is suggested that clinicians should not ignore other diseases that cause ground glass shadow in the lung during the current epidemic of COVID-19.
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Systemic juvenile idiopathic arthritis(sJIA) is one of the most serious critical illnesses in childhood, characterized by high fever, recurrent rash, and arthritis, etc.Children with sJIA associated-lung disease(sJIA-LD) are more severely ill and have a worse prognosis, the correlation between the mechanism and age, disease activity, anti-rheumatic drug therapy, applications of biologics, infection and other factors is worth exploring.This article reviews the research progress on the mechanism, risk factors, treatment methods and prognosis of sJIA-LD, so as to provide a theoretical basis for improving the diagnosis and treatment of sJIA and improving the prognosis.
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Resumen | Introducción. No se dispone de pruebas sensibles ni específicas para diagnosticar la artritis idiopática juvenil sistémica. Objetivo. Evaluar la utilidad diagnóstica de niveles de ferritina total cinco veces por encima del valor normal (ferritina total>5N) y el porcentaje disminuido (menor de o igual a 20 % de la ferritina total) de la ferritina glucosilada (ferritina glucosilada<20 %) para el diagnóstico de artritis idiopática juvenil sistémica en pacientes con fiebre de origen desconocido evaluados por reumatología pediátrica. Materiales y métodos. Se hizo un estudio observacional de pruebas diagnósticas de corte transversal en menores de 16 años hospitalizados entre el 2010 y el 2014. El patrón diagnóstico de referencia fue el cumplimiento de los criterios de clasificación o diagnóstico confirmado en el seguimiento. Se determinaron las medidas de utilidad de las pruebas. Resultados. Se incluyeron 40 pacientes con fiebre de origen desconocido: 11 con artritis idiopática juvenil sistémica y 29 con otros diagnósticos. La mediana de la ferritina total fue mayor en la artritis idiopática juvenil sistémica (3.992 ng/ml) comparada con otras causas de fiebre de origen desconocido (155 ng/ml) (p=0,0027), así como la ferritina total>5N (90,91 % Vs. 51,72 %) (p=0,023). El porcentaje de ferritina glucosilada≤20 % fue de 96,5 % en otras fiebres de origen desconocido en comparación con la artritis idiopática juvenil sistémica (81,8 %) (p=0,178). La ferritina total>5N tuvo una sensibilidad del 91 %, una especificidad del 48 %; un cociente de probabilidades (Likelihood Ratio, LR) positivo de 1,76 y uno negativo de 0,19, demostrando mayor utilidad para el diagnóstico que la combinación de la ferritina total>5N y ferritina glucosilada≤20 %, cuya sensibilidad fue del 81,8 %, la especificidad del 48,3 %, un cociente de probabilidades LR positivo de 1,58 y un LR negativo de 0,38. Conclusión. En pacientes con fiebre de origen desconocido evaluados por reumatología pediátrica, la ferritina total>5N demostró ser útil como prueba de tamización para el diagnóstico de artritis idiopática juvenil sistémica.
Abstract | Introduction: There are no sensitive or specific tests available to diagnose systemic juvenile idiopathic arthritis (sJIA). Objective: To assess the utility as diagnostic tests of total ferritin (TF) levels greater than 5 times the normal value (TF>5N) and the decreased percentage (less than or equal to 20% of TF) of glycosylated ferritin (GF≤20%) for the diagnosis of sJIA in patients with fever of unknown origin evaluated by pediatric rheumatology. Materials and methods: We conducted an observational, cross-sectional study of diagnostic tests in children under 16 years of age hospitalized between 2010 and 2014. The reference diagnostic standard was the fulfillment of the classification criteria or confirmed diagnosis at follow-up. We determined the measures of utility of the tests. Results: We included 40 patients with fever of unknown origin, 11 with sJIA, and 29 with other diagnoses. The median TF was higher in sAIJ (3992 ng/ml) versus other causes of fever of unknown origin (155 ng/ml) (p=0.0027), as well as TF>5N (90.91% versus 51.72%) (p=0.023). The percentage of GF≤20% was higher in patients with other causes of fever of unknown origin (96.5%) compared to sJIA (81.8%) (p=0.178). TF>5N had a sensitivity of 91%, specificity of 48%, positive likelihood ratio (LR) of 1.76, and negative LR of 0.19 demonstrating greater utility for the diagnosis of sJIA than the combination of FT> 5N with GFR <20%, with a sensitivity of 81.8%, specificity of 48.3%, positive LR of 1.58, and negative LR of 0.38. Conclusion: In patients with FUO evaluated by pediatric rheumatology, TF> 5N proved useful as a screening test for the diagnosis of sJIA.
Subject(s)
Arthritis, Juvenile/diagnosis , Ferritins , Odds Ratio , Sensitivity and SpecificityABSTRACT
RESUMEN La artritis idiopática juvenil sistémica, también conocida como enfermedad de Still, se considera un trastorno autoinflamatorio y suele ser la más compleja y grave entre todas las formas clínicas de la enfermedad. Cursa generalmente en forma de brotes de actividad repetidos, intercalados por periodos de remisión. Se presenta el caso de una paciente femenina de 4 años de edad, con diagnóstico de enfermedad de Still a los 2 años. Actualmente tiene tratamiento con triple terapia de inducción: cloroquina, metotrexato y salazosulfapiridina con actividad de la enfermedad persistentemente alta por JADAS 27. Acudió a consulta por presentar fiebre, toma del estado general y manifestaciones respiratorias de tres días de evolución que se interpretó como una infección respiratoria baja. Se prescribió tratamiento con antibióticos sin signos de mejoría. A los 7 días se agravó el cuadro clínico y se planteó el diagnóstico de síndrome de activación macrofágica. Se comenzó protocolo de tratamiento con esteroides en combinación con otros fármacos de probada eficacia para esta situación clínica (etopósido, ciclosporina, metotrexato). Se revaloró política de antibióticos sin lograrse respuesta satisfactoria y se decidió introducir el rituximab que aporta excelentes resultados. Después de 3 meses de difícil manejo, la paciente egresó del hospital recuperada de esta complicación y con bajo nivel de actividad de la enfermedad de base.
ABSTRACT Systemic Juvenile Idiopathic Arthritis, also known as Still's disease, is considered an autoinflammatory disorder and is often the most complex and severe of all clinical forms of the disease. It usually takes the form of repeated bouts of activity, interspersed with periods of remission. We present the case of a 4-year-old female patient, diagnosed with Still's disease at 2 years of age. Currently undergoing treatment with triple induction therapy: chloroquine, methotrexate and salazosulfapyridine with persistently high disease activity due to JADAS 27. He comes to the clinic due to fever, general condition, and respiratory manifestations of three days of evolution interpreted as an infectious respiratory process under. Antibiotic treatment is started without signs of improvement. At 7 days the clinical picture worsens, and the diagnosis of Macrophage Activation Syndrome is raised. A steroid treatment protocol is started in combination with other drugs of proven efficacy for this clinical situation (ethopside, cyclosporine, methotrexate). Antibiotic policy was reassessed without achieving a satisfactory response and it was decided to introduce rituximab, which provides excellent results. After three months of difficult management, the patient was released from the hospital recovered from this complication and with a low level of activity of the underlying disease.
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Objective:To investigate the clinical features, diagnosis and treatment of systemic juvenile idiopathic arthritis (SJIA) complicated with macrophage activation syndrome (MAS).Methods:From January 1st, 2018 to January 1st, 2020, 7 cases of SJIA-MAS were diagnosed. Their clinical and laboratory data were collected and summarized.Results:In these 7 cases, 2 were males and 5 were females, the ratio of male to female was 2∶5. The age range was 11 months to 2 years old. The course of disease was 14 to 32 days. The clinical manifestations included fever and rash in 7 without arthritis; hepatomegaly, splenomegaly and lymphadenopathy in 7; hematological involvement in 7; nervous system involvement in 2; digestive system involvement in 7; respiratory system involvement in 7; cardiovascular involvement in 3. White blood cell was decreased in 1 case, platelet was decreased in 1 case and hemoglobin was decreased in 7 cases. Ferritin, triglyceride, alanine transaminas and aspartate aminotransferase were increased in 7 cases, fibrinogen was significantly decreased in 7 cases, and direct bilirubin was increased in 4 cases. IL-2R was significantly increased. Hemophagocytosis was observed in bone marrow of 4 cases. Cerebrospinal fluid protein was 2 005 mg/L in 1 case. All the 7 cases were tested for exon genes, and no pathogenic mutation was found. All of the 7 cases showed lung lesions in chest CT scan. Multiple demyelinating lesions were found in 1 case by head magnetic resonance imaging. One case was treated with high-dose intravenous methylprednisolone combined with IL-6 receptor antagonist(tocilizumab). The other 6 cases were treated with high-dose intravenous methylprednisolone combined with cyclosporine A (CsA). Two cases were treated with Janus kinases inhibitor(tofacitinib). After treatment, 7 cases got relieved, no death, no recurrence oocurred during the follow-up.Conclusion:Acute onset, multiple organ involvement and no joint inflammation are prominent in MAS of infants and toddlers. High fever, proressive reduction of blood cells and increase of SF are significant in SJIA-MAS. High dose glucocorticoid combined with CsA can benefit in most cases, and some severe cases need to be treated with biological agents.
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Objective To detect the levels of procalcitonin in multiple genes autoinflammatory disease (adult Still disease,systemic juvenile idiopathic arthritis,crohn's Crohn's disease),and to explore the relationship between erythrocyte sedimentation rate (ESR),C-reactive protein (CRP),ESR/CRP and disease or complicated infection combined disease.Methods One hundred and fifty-three patients were en-rolled,88 patients with multiple genes autoinflammatory disease,including 32 cases of adult Still disease,27 cases of systemic juvenile idiopathic arthritis,29 cases of Crohn's disease.In addition,30 cases of healthy controls,35 patients with systemic lupus erythematosus (SLE) were included into this study.Electroche-miluminescence was used to test the value of serum PCT,erythrocyte sedimentation rate was tested by blood sedimentation instrument method,the CRP level was tested by lmmunoturbidimetry,and the data was handed managed and analysised by matlab software and One-way analysis of variance (ANOVA) was used to compare the differences of PCTs between groups.Results ① In the non-infection condition,the PCT value of the autoimmune inflammatory diseases [(0.36±0.74) μg/L,95％confidence interval (CI) (0.174 9,0.550 9) μg/L)] was signifieantly higher than that of the healthy control group [(0.06±0.06) μg/L,95％CI (0.035 1,0.081 7 μg/L)],the difference was statistically significant (F=5.03,P=0.027 4),but there was no statistically significant (F=1.03,P=0.475 5) when comparing with SLE group.② The PCT level of the non-infected inflammatory enteric arthritis [(0.20±0.32),95％CI(0.042 7,0.364 3) μg/L] was different compared with the healthy group,the difference was statistically significant (F=5.77,P=0.020 4),at the same time,the difference was not statistically significant when comparing with the SLE group (F=0.22,P=0.647 6).When the PCT value in non-infected adults Still disease [(0.60±1.02) 95％CI(0.048 4,1.153 6) μg/L] compared with the healthy group,the difference was statistically significant (F=7.22,P=0.01) but the difference was not statistically different when compared with the SLE group (F=2.65,P=0.114 3).The PCT level difference was statistically significant (F=2.23,P＜0.01)when comparing infection-free juvenile idiopathic arthritis [(1.52±2.02) μg/L,95％CI(0.054 8,4.591 9) μg/L] and the healthy group,the difference was statistically significantly different (F=8.34,P=0.004 7) when compared with the PCT of the non-infected SLE group.③ In the case of autoinflammatory diseases without infection,the 95％CI of ESR/CRP ratio was between 1.121 2 and 3.589 4.In the case of co-infection,the 95％ CI of ESR/CRP ratio was between 1.502 2 and 8.718 8,so we considered autoimmune inflammatory diseases might had a high possibility of co-infection when the ESR/CRP ratio was higher than 3.5.Conclusion ① The multiple genes autoinflammatory disease group has a higher value of PCT level than healthy controls even without infection.② The mean and 95％CI range of PCT of the inflammatory bowel disease arthritis,adult Still disease and the juvenile id-iopathic arthritis is significantly higher than the healthy controls,partially higher than SLE group.In addition,the PCT level in the juvenile idiopathic arthritis is the highest.③ In clinical,to estimate whether the multiple genes autoinflammatory disease has bacterial infection,we can't just simply rely on PCT to estimate whether the multiple genes autoinflammatory disease has bacterial infection,we may consider the ratio of the ESR/CRP,when the value is higher than 3.5,we may consider patients has strong probability with infection.
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Indication of glucocorticoid use in juvenile idiopathic arthritis (JIA)are:non - steroidal anti - in-flammatory drug invalid systemic JIA,macrophage activation syndrome,complicated with inflammatory bowel disease, complicated with uveitis,intra - articular injection in patients with active arthritis. Due to the long - term use of glu-cocorticoids with various risk of side effects. It is necessary to closely regularly monitor the side effects of glucocorti-coids,strictly control the dosage and course,and add traditional and/ or biological disease - modifying anti - rheumatic drugs in time.
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Systemic juvenile idiopathic arthritis (sJIA) is a systemic inflammatory disease characterized with arthritis in one or more joints,fever,rash and serositis.Due to the atypical symptoms and poor prognosis,it's a great challenge in the clinical diagnosis and treatment efficacy evaluation in sJIA.These challenges could be addressed by the identification of clinical biomarkers,aiming at evaluating the disease severity,predicting the activity and prognosis of sJIA.This review will be focus on genetics,serum and cellular markers in sJIA and summarize the relative application on diagnosis and treatment.
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Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory disorder characterized by fever,lymphadenopathy,arthritis,rash and serositis.In sJIA,systemic inflammation has been associated with dysregulation of the innate immune system,suggesting that it is an autoinflammatory disorder.Interleukin(IL)-1 and IL-6 play a major role in the pathogenesis of sJIA.Glucocorticoids and disease modifying anti-rheumatic drugs(DMARDS) are the conventional treatment of patients with sJIA.The major advances in the therapy of sJIA are the applications of IL-1 and IL-6 inhibitors and have shown to be highly effective.Recent data suggests that early cytokine blockage might alter the chronic arthritis course,which reflect a potential window of opportunity in the care of children with sJIA.The purpose of this article is to discuss the treatment approach of the patients with sJIA according to the recently published literature.
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Objective To summarize the treatment experience of refractory systemic juvenile idiopathic arthritis (JIA) by tocilizumab, and to explore the cost-effective treatment. Methods The clinical data of 6 pediatric patients with refractory systemic JIA treated by tocilizumab from 2014 to June 2016 were retrospectively analyzed in the aspects of course and effectiveness of tocilizumab, steroid reduction, adverse reaction, and growth. Results The median age of the six patients (3 males and 3 females) was 6 years, and the course of disease were from 16 to 63 months. All patients were treated by other immunosuppressive agents or biological agents in addition to steroid and traditional anti-rheumatic drug therapy. The courses of tocilizumab treatment were from 7 to 26 months and the median time was 9.5 months. All 6 patients responded to tocilizumab and achieved the clinical remission at different time. After the induced remission, the interval of the treatment intervention was increased from 2 weeks up to 4 weeks in 3 cases, and no disease activity was observed. Except one case, another 5 cases reduced and stopped the use of hormones at 5.8 months after tocilizumab treatment. After hormones was reduced and discontinued, the growth was improved. All 6 patients had no serious adverse reactions. Conclusions Tocilizumab is safe and effective for patients with refractory JIAs. The steroid can be reduced in short time to improve growth. After remission is induced, the interval of the treatment intervention could be prolonged.
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Juvenile idiopathic arthritis (JIA)is one of the most common chronic rheumatic diseases in childhood.Systemic juvenile idiopathic arthritis(SJIA) is a subtype of JIA,which may be different from other subtypes.As shown in studies,interleukin-6 (IL-6),IL-18,IFN-γ,may instead of TNF-α,play an important role in SJIA.Traditional biological agents such as TNF antagonists have limited effect in the patients with SJIA.Tocilizumab(TCZ),a humanized anti-IL-6 receptor monoclonal antibody,inhibits the binding of IL-6 with transmembrane IL-6R or soluble IL-6R,blocking the IL-6 mediated inflammation and joint destruction.This article reviews the efficacy and safety of TCZ in patients with SJIA.
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Objective To evaluate the value of soluble interleukin 2 receptor (sIL-2R),interleukin (IL)-6,IL-10 and tumor necrosis factor-cα (TNF-α) used in differential diagnosis of systemic juvenile idiopathic arthritis (sJIA) and sepsis in children.Methods Each of 41 children with sJIA and sepsis were enrolled and 41 healthy children were selected as the healthy control group at Children's Hospital of Nanjing Medical University from January 2015 to July 2017.Levels of serum sIL-2R,IL-6,IL-10,TNF-α and peripheral blood levels of white blood cells (WBC),hemoglobin (Hb),platelets (PLT),high sensitivity C-reactive protein (hs-CRP),erythrocyte sedimentation rate (ESR) and procalcitonin (PCT) were compared between the sJIA group and sepsis group before treatment and those of the healthy control group.Results Peripheral blood levels of WBC,Hb,PLT,CRP,ESR and PCT in the healthy control group were (8.43 ± 2.35) × 109/L,(124.46 ± 8.76) g/L,(298.45 ± 100.23) × 109/L,(11.32 ± 5.76) mg/L,(18.32 ± 9.33) mm/1 h,(0.27±0.17) μg/L and the levels of serum sIL-2R,IL-6,IL-10,TNF-α were (483.24 ± 255.33) kU/L,(7.38 ±4.02) ng/L,(5.45 ± 3.06) ng/L,(8.23 ± 3.03) ng/L,respectively.Peripheral blood levels of WBC,Hb,PLT,CRP,ESR and PCT in the sJIA group before treatment were (17.53 ± 7.98) × 109/L,(105.76 ± 13.33) g/L,(389.43 ± 176.34) × 109/L,(88.32 ± 43.21) mg/L,(55.32 ± 34.23) mmn/1 h,(0.53 ±0.24) μg/L and the levels of serum sIL-2R,IL-6,IL-10,TNF-α were (945.35 ±436.75) kU/L,(132.39 ± 58.43) ng/L,(7.02 ± 5.12) ng/L,(14.32 ± 7.95) ng/L.Peripheral blood levels of WBC,Hb,PLT,CRP,ESR and PCT in sepsis group before treatment were (16.88 ± 6.54) × 109/L,(102.95 ± 20.18) g/L,(302.87 ± 124.45) × 109/L,(60.41 ± 33.24) mg/L,(53.75 ± 28.43) mm/1 h,(2.84 ± 1.76) μg/L and the levels of serum sIL-2R,IL-6,IL-10,TNF-α were (2 476.36 ± 1 574.11) kU/L,(39.47 ± 20.32) ng/L,(6.31 ±4.04) ng/L,(15.87 ±6.32) ng/L.Blood levels of WBC,CRP,ESR,PCT,sIL-2R,IL-6 and TNF-α in the sJIA and the sepsis group were significantly higher than those in healthy control group.Serum levels of sIL-2R and PCT in the sJIA group were obviously lower than those in the sepsis group,but the level of IL-6 was higher than that in the sepsis group.Conclusion Combined detection of blood sIL-2R,IL-6 and PCT can provide a reference and reduce mistaken diagnosis of sJIA and sepsis in the earlier clinical stage.
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Objective To explore the efficacy and safety of Tocilizumab in the treatment of systemic juvenile idiopathic arthritis (sJIA)in children.Methods Twenty-four sJIA patients were collected who were hospitalized at the Department of Rheumatology and Immunity,Jiangxi Provincial Children's Hospital from October 2015 to May 2016,and they received Tocilizumab combined with Methotrexate (MTX) treatment for 12 weeks.The clinical laboratory and physiological indices,including routine blood,liver and kidney function tests,number of joints with active arthritis,number of joints with limited range of motion,physicians and patients assessment of disease activity,childhood health questionnaire,erythrocyte sedimentation rate(ESR),C-reactive protein (CRP),and compliance rates of Pediatrics of American College of Rheumatology(ACR Ped) 30,50,70 were observed after 4,8 and 12 weeks of treatment,and the adverse reactions were recorded.Results After 4 weeks of treatment,the levels of white blood cells,platelet,ESR and CRP in 24 cases of sJIA significantly decreased compared with those of the patients before treatment [(15.1 ± 2.7) × 109/L vs.(24.2 ±3.5) × 109/L,(277 ±73) × 109/L vs.(368 ± 62) × 109/L,(25 ± 12) mm/1 h vs.(75 ± 15) mm/1 h,(20 ± 13) mg/L vs.(64 ± 1) mg/L],and the differences were statistically significant (t =10.08,4.65,70.71,26.78,all P ＜0.05);the hemoglobin was increased dramatically[(110 ± 12) g/L vs.(98 ± 10) g/L],and the difference was statistically significant(t =-3.76,P ＜ 0.05).The compliance rates of ACR Ped 30,50,70 after 4 weeks of treatment were 82％,74％,68％,and they were continuously improved after 8 weeks of treatment (90％,82％,78％)and 12 weeks of treatment (98％,93％,92％),and the differences were all statistically significant (F =7.11,7.29,8.86,all P ＜0.05).The levels of IL-6 after 12 weeks of treatment had no significant change compared with those of the patients pre-treatment [(10.8 ±2.5) ng/L vs.(12.7 ±3.0) ng/L,t =1.96,P ＞0.05].Conclusion Tocilizumab is effective and safe in the treatment of sJIA patients,which can improve the symptoms,signs and laboratory inflammatory activity indexes of sJIA in a short time.
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The typical clinical manifestations of systemic juvenile idiopathic arthritis (sJIA) are fever,rash,arthralgia,polyserositis,hepatomegaly and/or splenomegaly.It is a subtype of JIA,the morbidity is 6.6/100 000-15.0/100 000,but the disability and mortality account for more than 2/3 of JIA.Its symptoms and signs are often not quite typical,therefore,it is often difficult to diagnose.The pathogenesis of sJIA is unclear at present,it is considered to be a self inflammatory response syndrome,rather than classical autoimmune arthritis.But,self inflammatory disease has a clear pathogenic gene and family history,the pathogenic gene of sJIA is still inconclusive,it also doesn't have obvious familial inheritance,this is the greatest difference.The traditional treatment for sJIA includes non steroidal anti-inflammatory drugs,glucocorticoids and disease modifying antirheumatic drugs.But,it is still dangerous and easy to relapse,what's more,it is prone to result in fatal complications-macrophage activation syndrome.This article will review the etiology,pathogenesis,clinical manifestations,diagnosis,new biomarkers,differential diagnosis and treatment of sJIA.
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Systemic juvenile idiopathic arthritis (sJIA) is systemic inflammatory disease classified as a subtype of juvenile idiopathic arthritis (JIA).Besides arthritis,it is characterised by systemic features such as spiking fever,skin rash,hepatosplenomegaly or serositis.It is becoming clear now that abnormalities in the innate immunity [cytokines such as interleukin (IL)-1,IL-6 and IL-18,and neutrophils and monocytes/macrophages rather than lymphocytes] play a major role in the pathogenesis of sJIA,distinguishing it from other JIA subtypes.Another distinctive feature of sJIA is its strong association with macrophage activation syndrome (MAS).Based on this,consensus is emerging that sJIA should be viewed as an autoinflammatory syndrome rather than a classic auto-immune disease.As a consequence of the progression in understanding the underlying mechanisms of sJIA,major changes in the management are evolving.Recently,remarkable improvement has been observed with IL-1 and IL-6 targeted therapies.These therapies might also change the long-term outcome of this disease.
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Objective To investigate clinical characteristics of refractory systemic juvenile idiopathic arthritis(JIA)and the efficiency of glucocorticoid in therapy on this kind of disease.Methods Thirty-nine children with systemic JIA were divided into low dose group 0.5-1.0 mg/(kg?d)and high dose group 1.0-1.5 mg/(kg?d).And the efficiency was observed by change of active index after 10 and 20 days.Results The effective power was 58.8% and 72.7% after 10 days,respectively.After 20 days,the power was 76.5% and 90.9%,respectively.The power in high dose group was significantly higher than that in low dose group.It had no difference in statistical analysis for efficiency of 2 kind of glucocorticoid dosage to control fever,but it had obvious difference to control arthralgia,arthrocele,erythrocyte sedimentation rate(ESR),C-reactive protein(CRP).Conclusion Glucocorticoid therapy is very effective to control the activity of disease in patients with systemic JIA.