Background Immunoglobulin A vasculitis(IgAV)is the most common cause of systemic vasculitis in childhood.Due to the continued use of the disease name"anaphylactoid purpura"in China,several misunderstandings ...Background Immunoglobulin A vasculitis(IgAV)is the most common cause of systemic vasculitis in childhood.Due to the continued use of the disease name"anaphylactoid purpura"in China,several misunderstandings have arisen in clinical prac-tice and treatment regimens differ widely.In addition,new research and evidence-based data have grown.The Subspecialty Group of Immunology,Society of Pediatrics,Chinese Medical Association and the Chinese Alliance of Pediatric Rheumatic and Immunologic Diseases initiated an update of guidelines for the diagnosis and management of childhood IgAV.The aim therefore was to provide agreed consensus recommendations for diagnosis and treatment for children with IgAV. Methods This study utilized the Delphi technique to develop an evidence-based expert consensus for childhood IgAV.We conducted a systematic literature review to retrieve evidence,which was graded using GRADE(Grading of Recommenda-tions Assessment,Development,and Evaluation)criteria.Two rounds of Delphi voting and a consensus meeting involving 23 experts were conducted.Recommendations were accepted when ≥75%of experts agreed. Results In total,five recommendations for diagnosis,six for treatment,one for prognosis,and two for health education for pediatric IgAV were accepted.Diagnostic recommendations included the use of the European Alliance of Associations for Rheumatology/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society-endorsed Ankara 2008 classification criteria for IgAV diagnosis.In addition,appropriate use of imaging,gastrointestinal endoscopy,skin biopsy,and kidney biopsy are recommended.Kidney biopsy is recommended for children with IgAV pre-senting with nephrotic syndrome/nephrotic-range proteinuria,reduced estimated glomerular filtration rate,acute nephritis syndrome,or persistent moderated/mild proteinuria.Treatment recommendations involved indications for glucocorticoid use,immunosuppressant use,and intravenous immunoglobulin use.It also addressed the inappropriate use of prophylactic glucocorticoid treatment and recommended against routine employment of plasmapheresis.Health education placed emphasis on the inappropriate use of anti-anaphylactic treatment and proffers dietary suggestions. Conclusions This guideline provides evidence-based recommendations for the diagnosis and management of IgAV in chil-dren.This will facilitate improved and standardized care.展开更多
Background Systemic lupus erythematosis(SLE)is a complex and clinically heterogeneous autoimmune disease.A variety of immunological defects contribute to SLE,including dysregulated innate and adaptive immune response....Background Systemic lupus erythematosis(SLE)is a complex and clinically heterogeneous autoimmune disease.A variety of immunological defects contribute to SLE,including dysregulated innate and adaptive immune response.A clearer understanding of the mechanisms driving disease pathogenesis combined with recent advances in medical science is predicted to enable accelerated progress towards improved SLE-personalized approaches to treatment.The aim of this review was to clarify the immunological pathogenesis and treatment of SLE.Data sources Literature reviews and original research articles were collected from database,including PubMed and Wanfang.Relevant articles about SLE were included.Results Breakdown of self-tolerance is the main pathogenesis of SLE.The innate and adaptive immune networks are interlinked with each other through cytokines,complements,immune complexes and kinases of the intracellular machinery.Treatments targeted at possible targets of immunity have been assessed in clinical trials.Most of them did not show better safety and efficacy than traditional treatments.However,novel targeting treatments are still being explored.Conclusions Dysregulated immune response plays a critical role in SLE,including innate immunity and adaptive immunity.Biologic agents that aim to specifically target abnormal immune processes were assessing and may bring new hope to SLE patients.展开更多
Background Macrophage activation syndrome(MAS)is a major cause of morbidity and mortality in pediatric rheumatology.We aimed to further understand the clinical features,treatment,and outcome of MAS in China.Methods A ...Background Macrophage activation syndrome(MAS)is a major cause of morbidity and mortality in pediatric rheumatology.We aimed to further understand the clinical features,treatment,and outcome of MAS in China.Methods A multi-center cohort study was performed in seven hospitals in China from 2012 to 2018.Eighty patients with MAS were enrolled,including 53 cases with systemic juvenile idiopathic arthritis(SJIA-MAS),10 cases of Kawasaki disease(KD-MAS),and 17 cases of connective tissue disease(CTD-MAS).The clinical and laboratory data were collected before(pre-),at onset,and during full-blown stages of MAS.We compared the data among the SJIA-MAS,KD-MAS,and CTD-MAS subjects.Results 51.2%of patients developed MAS when the underlying disease was first diagnosed.In patients with SJIA,22.6%(12/53)were found to have hypotension before the onset of SJIA-MAS.These patients were also found to have significantly increased aspartate aminotransferase(AST)and lactate dehydrogenase(LDH),as well as decreased albumin(P<0.05),but no difference in alanine aminotransferase,ferdtin,and ratio of ferritin/erythrocyte sedimentation rate(ESR)at onset of MAS when compared to pre-MAS stages of the disease.In addition,ferritin and ratio of ferritin/ESR were significantly elevated in patients at full-blown stages of SJIA-MAS compared to pre-MAS stage.Significantly increased ferritin and ratio of ferritin/ESR were also observed in patients with SJIA compared to in KD and CTD.Receiver-operating characteristic analysis showed that 12,217.5μg/L of ferritin and 267.5 of ferritin/ESR ratio had sensitivity(80.0%and 90.5%)and specificity(88.2%and 86.7%),respectively,for predicting full-blown SJIA-MAS.The majority of the patients received corticosteroids(79/80),while biologic agents were used in 12.5%(10/80)of cases.Tocilizumab was the most commonly selected biologic agent.The overall mortality rate was 7.5%.Conclusions About half of MAS occurred when the underlying autoimmune diseases(SJIA,KD,and CTD)were first diagnosed.Hypotension could be an important manifestation before MAS diagnosis.Decreased albumin and increased AST,LDH,ferritin,and ratio of ferritin/ESR could predict the onset or full blown of MAS in patient with SJIA.展开更多
基金supported by the National Key Research and Development Program of China(2021YFC2702004).
文摘Background Immunoglobulin A vasculitis(IgAV)is the most common cause of systemic vasculitis in childhood.Due to the continued use of the disease name"anaphylactoid purpura"in China,several misunderstandings have arisen in clinical prac-tice and treatment regimens differ widely.In addition,new research and evidence-based data have grown.The Subspecialty Group of Immunology,Society of Pediatrics,Chinese Medical Association and the Chinese Alliance of Pediatric Rheumatic and Immunologic Diseases initiated an update of guidelines for the diagnosis and management of childhood IgAV.The aim therefore was to provide agreed consensus recommendations for diagnosis and treatment for children with IgAV. Methods This study utilized the Delphi technique to develop an evidence-based expert consensus for childhood IgAV.We conducted a systematic literature review to retrieve evidence,which was graded using GRADE(Grading of Recommenda-tions Assessment,Development,and Evaluation)criteria.Two rounds of Delphi voting and a consensus meeting involving 23 experts were conducted.Recommendations were accepted when ≥75%of experts agreed. Results In total,five recommendations for diagnosis,six for treatment,one for prognosis,and two for health education for pediatric IgAV were accepted.Diagnostic recommendations included the use of the European Alliance of Associations for Rheumatology/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society-endorsed Ankara 2008 classification criteria for IgAV diagnosis.In addition,appropriate use of imaging,gastrointestinal endoscopy,skin biopsy,and kidney biopsy are recommended.Kidney biopsy is recommended for children with IgAV pre-senting with nephrotic syndrome/nephrotic-range proteinuria,reduced estimated glomerular filtration rate,acute nephritis syndrome,or persistent moderated/mild proteinuria.Treatment recommendations involved indications for glucocorticoid use,immunosuppressant use,and intravenous immunoglobulin use.It also addressed the inappropriate use of prophylactic glucocorticoid treatment and recommended against routine employment of plasmapheresis.Health education placed emphasis on the inappropriate use of anti-anaphylactic treatment and proffers dietary suggestions. Conclusions This guideline provides evidence-based recommendations for the diagnosis and management of IgAV in chil-dren.This will facilitate improved and standardized care.
文摘Background Systemic lupus erythematosis(SLE)is a complex and clinically heterogeneous autoimmune disease.A variety of immunological defects contribute to SLE,including dysregulated innate and adaptive immune response.A clearer understanding of the mechanisms driving disease pathogenesis combined with recent advances in medical science is predicted to enable accelerated progress towards improved SLE-personalized approaches to treatment.The aim of this review was to clarify the immunological pathogenesis and treatment of SLE.Data sources Literature reviews and original research articles were collected from database,including PubMed and Wanfang.Relevant articles about SLE were included.Results Breakdown of self-tolerance is the main pathogenesis of SLE.The innate and adaptive immune networks are interlinked with each other through cytokines,complements,immune complexes and kinases of the intracellular machinery.Treatments targeted at possible targets of immunity have been assessed in clinical trials.Most of them did not show better safety and efficacy than traditional treatments.However,novel targeting treatments are still being explored.Conclusions Dysregulated immune response plays a critical role in SLE,including innate immunity and adaptive immunity.Biologic agents that aim to specifically target abnormal immune processes were assessing and may bring new hope to SLE patients.
基金This study is funded by Zhejiang Basic Public Welfare Research Project(LGF19H100002).
文摘Background Macrophage activation syndrome(MAS)is a major cause of morbidity and mortality in pediatric rheumatology.We aimed to further understand the clinical features,treatment,and outcome of MAS in China.Methods A multi-center cohort study was performed in seven hospitals in China from 2012 to 2018.Eighty patients with MAS were enrolled,including 53 cases with systemic juvenile idiopathic arthritis(SJIA-MAS),10 cases of Kawasaki disease(KD-MAS),and 17 cases of connective tissue disease(CTD-MAS).The clinical and laboratory data were collected before(pre-),at onset,and during full-blown stages of MAS.We compared the data among the SJIA-MAS,KD-MAS,and CTD-MAS subjects.Results 51.2%of patients developed MAS when the underlying disease was first diagnosed.In patients with SJIA,22.6%(12/53)were found to have hypotension before the onset of SJIA-MAS.These patients were also found to have significantly increased aspartate aminotransferase(AST)and lactate dehydrogenase(LDH),as well as decreased albumin(P<0.05),but no difference in alanine aminotransferase,ferdtin,and ratio of ferritin/erythrocyte sedimentation rate(ESR)at onset of MAS when compared to pre-MAS stages of the disease.In addition,ferritin and ratio of ferritin/ESR were significantly elevated in patients at full-blown stages of SJIA-MAS compared to pre-MAS stage.Significantly increased ferritin and ratio of ferritin/ESR were also observed in patients with SJIA compared to in KD and CTD.Receiver-operating characteristic analysis showed that 12,217.5μg/L of ferritin and 267.5 of ferritin/ESR ratio had sensitivity(80.0%and 90.5%)and specificity(88.2%and 86.7%),respectively,for predicting full-blown SJIA-MAS.The majority of the patients received corticosteroids(79/80),while biologic agents were used in 12.5%(10/80)of cases.Tocilizumab was the most commonly selected biologic agent.The overall mortality rate was 7.5%.Conclusions About half of MAS occurred when the underlying autoimmune diseases(SJIA,KD,and CTD)were first diagnosed.Hypotension could be an important manifestation before MAS diagnosis.Decreased albumin and increased AST,LDH,ferritin,and ratio of ferritin/ESR could predict the onset or full blown of MAS in patient with SJIA.
