A Artrite Idiopática Juvenil Sistêmica é marcada pela gravidade dos sintomas que aparecem fora das articulações (como febre e manchas na pele) e por afetar igualmente meninos e meninas.
Introdução
O que você precisa saber de cara
A Artrite Idiopática Juvenil Sistêmica é marcada pela gravidade dos sintomas que aparecem fora das articulações (como febre e manchas na pele) e por afetar igualmente meninos e meninas.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 9 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 22 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
4 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial, Unknown.
The anti-Muellerian hormone (AMH) plays an important role in several reproductive functions (PubMed:14742691, PubMed:34155118, PubMed:3754790, PubMed:8469238). Anti-Muellerian hormone binds and activates AMHR2, its specific type-II receptor, that heterodimerizes with type-I receptors (ACVR1 and BMPR1A) to regulate target gene expression through downstream SMAD protein signal transduction (PubMed:20861221, PubMed:34155118). Produced and secreted by Sertoli cells of the male fetus, anti-Muellerian
Secreted
Persistent Muellerian duct syndrome 1
A form of male pseudohermaphroditism characterized by a failure of Muellerian duct regression in otherwise normal males.
A beta chain of antigen-presenting major histocompatibility complex class II (MHCII) molecule. In complex with the alpha chain HLA-DRA, displays antigenic peptides on professional antigen presenting cells (APCs) for recognition by alpha-beta T cell receptor (TCR) on HLA-DRB1-restricted CD4-positive T cells. This guides antigen-specific T-helper effector functions, both antibody-mediated immune response and macrophage activation, to ultimately eliminate the infectious agents and transformed cells
Cell membraneEndoplasmic reticulum membraneLysosome membraneLate endosome membraneAutolysosome membrane
Purine nucleoside enzyme that catalyzes the phosphorolysis of adenosine, guanosine and inosine nucleosides, yielding D-ribose 1-phosphate and the respective free bases, adenine, guanine and hypoxanthine (PubMed:31978345). Also catalyzes the phosphorolysis of S-methyl-5'-thioadenosine into adenine and S-methyl-5-thio-alpha-D-ribose 1-phosphate (PubMed:31978345). Also has adenosine deaminase activity (PubMed:31978345). Acts as a regulator of innate immunity in macrophages by modulating the purine
CytoplasmNucleusEndoplasmic reticulumPeroxisome
Juvenile arthritis
A rare, familial form of juvenile arthritis characterized by autosomal recessive inheritance and onset in early childhood of symmetric, chronic joint inflammation. It causes joint swelling, pain, stiffness and restricted joint movement. JUVAR has high clinical variability. Some patients exhibit systemic symptoms, including quotidian fever, erythematous rash, generalized lymphadenopathy, hepatomegaly, and/or splenomegaly. Others display polyarthritis without systemic inflammation.
Cytokine with a wide variety of biological functions in immunity, tissue regeneration, and metabolism. Binds to IL6R, then the complex associates to the signaling subunit IL6ST/gp130 to trigger the intracellular IL6-signaling pathway (Probable). The interaction with the membrane-bound IL6R and IL6ST stimulates 'classic signaling', whereas the binding of IL6 and soluble IL6R to IL6ST stimulates 'trans-signaling'. Alternatively, 'cluster signaling' occurs when membrane-bound IL6:IL6R complexes on
Secreted
Rheumatoid arthritis systemic juvenile
An inflammatory articular disorder with systemic onset beginning before the age of 16. It represents a subgroup of juvenile arthritis associated with severe extraarticular features and occasionally fatal complications. During active phases of the disorder, patients display a typical daily spiking fever, an evanescent macular rash, lymphadenopathy, hepatosplenomegaly, serositis, myalgia and arthritis.
Variantes genéticas (ClinVar)
118 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
20 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Artrite juvenil idiopática de início sistêmico
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
🟢 Recrutando agora
7 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
54 ensaios clínicos encontrados, 9 ativos.
Publicações mais relevantes
Systemic Onset Juvenile Idiopathic Arthritis in Sickle Cell Anaemia: A Diagnostic Odyssey.
An abnormal immune response masquerading as infectious conditions.
Kikuchi-Fujimoto disease (KFD) is a rare, benign, self-limited lymphadenitis most commonly affecting children and young adults, particularly females of Asian descent. Its etiology is unclear but is thought to involve immune dysregulation triggered by infection. We report a 14-year-old South Asian female presenting with 8 days of high fever, frontal headache, bilateral eye redness, transient arthralgias, and palpable lymphadenopathy in the cervical, axillary, and supraclavicular regions. Her history included recent travel to multiple countries and a prior episode of fever of unknown origin attributed to Bartonella. Initial laboratory evaluation revealed pancytopenia, elevated transaminases, and increased erythrocyte sedimentation rate and lactate dehydrogenase with normal C reactive protein. Infectious workup was negative for malaria, cytomegalovirus, West Nile virus, Rickettsia, Bartonella, and dengue. Ultrasound of the left axilla and computed tomography showed bilateral axillary lymphadenopathy. A cytokine panel demonstrated markedly elevated IL-18. Definitive diagnosis was established by axillary lymph node biopsy, which revealed histiocytic necrotizing lymphadenitis consistent with KFD. Systemic inflammatory disorders such as systemic-onset juvenile idiopathic arthritis, multisystem inflammatory syndrome in children, and Kawasaki disease were considered and excluded based on clinical, laboratory, and histopathological findings. The patient's fever resolved after dexamethasone administration, supporting an immune-mediated process. She subsequently developed recurrent fever, rash, and oral ulcers, which responded rapidly to intravenous corticosteroids, followed by a steroid taper.
Rescue Therapy With Continuous Intravenous Anakinra Infusion and Plasma Exchange in Refractory Cytokine Storm of Systemic JIA: A Case Report.
Macrophage activation syndrome (MAS) is one of the most common fatal complications of inflammatory diseases in children. In recent studies, Interleukin 1 inhibitors have been shown to be effective in suppressing the cytokine storm in MAS. There is limited literature describing the role of plasmapheresis in the cytokine storm syndromes as in MAS. A six-year-old boy presented with a 1-month history of daily fever, arthritis, cervical lymphadenopathy, skin rash, and elevated inflammatory markers. After multidisciplinary evaluation, he was diagnosed with systemic onset Juvenile Idiopathic Arthritis (sJIA) and started on IV methylprednisolone and SC anakinra. He later developed status epilepticus, required intubation, and progressed to multiorgan failure, needing inotropes and dialysis. Following a brief afebrile period, he worsened again, and labs confirmed MAS relapse with ferritin levels peaking at 26,000. Escalated treatment included switching to IV continuous anakinra, adding ciclosporin, and starting plasmapheresis. After three sessions of plasma exchange, he significantly improved, was extubated, and weaned off inotropes. However, attempts to switch anakinra back to SC failed twice, with recurrence of fever, rash, and elevated ferritin. He remained in PICU on IV continuous anakinra for over 3 weeks. When ferritin dropped to 900, an IV bolus regimen was trialed for a week, followed by a successful switch to SC anakinra. The child was discharged home asymptomatic with normalized labs. Anakinra has been used off-label in critically ill patients with MAS, thrombocytopenia, subcutaneous edema, and neurological dysfunction. Tapering from intravenous Anakinra to subcutaneous was challenging. This case taught us that tapering must begin at the right time to avoid hiccups or unfavorable outcomes. As reported in a few studies, plasmapheresis combined with immunosuppressants can reduce MAS-associated mortality by rapidly removing cytokines from the body.
Tofacitinib as an adjuvant treatment for pediatric Still's disease.
To describe the efficacy and safety of tofacitinib for pediatric Still's disease, also referred to as systemic-onset juvenile idiopathic arthritis (sJIA). Traditional non-biological drugs and drugs targeting the interleukin-1 and interleukin-6 pathways benefit some patients, but others show inadequate responses. We retrospectively analyzed ten patients with pediatric Still's disease who were treated with tofacitinib and had at least one follow-up visit. Data on patient history, laboratory findings, and treatments were collected at disease onset, at the initiation of tofacitinib, and during follow-up. Tofacitinib led to complete remission in six patients, partial remission in three patients, and loss of efficacy in one patient. Among the nine patients with remission, two discontinued corticosteroids entirely and seven used lower dosages of corticosteroids; these patients also used fewer concurrent medications (1 or 2) after tofacitinib initiation. The one patient who experienced loss of efficacy continued to require a higher dosage of corticosteroids and received five different additional medications. The other nine patients received tocilizumab and had a decreased frequency of these injections after tofacitinib initiation. Tofacitinib was well-tolerated, with only one reported instance of an upper respiratory tract infection. Tofacitinib appears to be an effective adjunct therapy for management of pediatric Still's disease, particularly for patients with unstable clinical conditions and adverse reactions to corticosteroids.
Acute lymphoblastic leukaemia presenting as chronic recurrent multifocal osteomyelitis.
A pre-schooler, born out of a non-consanguineous marriage, presented with on-and-off fever for 9 months, fleeting large joint swelling involving multiple joints for 5 months, weakness and pain in different limbs for 5 months, and difficulty in breathing for 2 weeks, 3 months ago. Initial differentials considered were haematogenous osteomyelitis and systemic-onset juvenile idiopathic arthritis. There was no rash, lymphadenopathy or hepatosplenomegaly. The initial bone marrow biopsy showed chronic inflammation and normal haematopoietic cells. The child developed septic arthritis in the right knee during hospitalisation. After excluding malignancy, chronic recurrent multifocal osteomyelitis was diagnosed, and anti-inflammatory medication was prescribed. Subsequently, the child exhibited pancytopenia and hepatosplenomegaly 2 months after hospital stay, and further bone marrow biopsy confirmed acute lymphoblastic leukaemia. This case underscores the importance of maintaining a vigilant approach to leukaemia when children present with musculoskeletal symptoms, as early detection and treatment are crucial for improving outcomes in childhood acute leukaemia.
Publicações recentes
Effectiveness of Canakinumab for First-Line Steroid-Free Treatment in Systemic-Onset Juvenile Idiopathic Arthritis and Juvenile Still Disease.
An abnormal immune response masquerading as infectious conditions.
Systemic Onset Juvenile Idiopathic Arthritis in Sickle Cell Anaemia: A Diagnostic Odyssey.
Rescue Therapy With Continuous Intravenous Anakinra Infusion and Plasma Exchange in Refractory Cytokine Storm of Systemic JIA: A Case Report.
Tofacitinib as an adjuvant treatment for pediatric Still's disease.
📚 EuropePMC201 artigos no totalmostrando 119
An abnormal immune response masquerading as infectious conditions.
SAGE open medical case reportsSystemic Onset Juvenile Idiopathic Arthritis in Sickle Cell Anaemia: A Diagnostic Odyssey.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionRescue Therapy With Continuous Intravenous Anakinra Infusion and Plasma Exchange in Refractory Cytokine Storm of Systemic JIA: A Case Report.
Case reports in pediatricsTofacitinib as an adjuvant treatment for pediatric Still's disease.
Frontiers in pediatricsSystemic juvenile idiopathic arthritis, with tinea capitis and peripheral eosinophilia: a case report.
Annals of medicine and surgery (2012)Endogenous Lipoid Pneumonia in Adult Autoinflammatory Disease.
Arthritis care & researchFlagellate Dermatitis in Systemic-Onset Juvenile Idiopathic Arthritis: A Rare Cutaneous Manifestation of an Autoinflammatory Storm.
Indian journal of pediatricsAcute lymphoblastic leukaemia presenting as chronic recurrent multifocal osteomyelitis.
BMJ case reportsThe Implications of Musculoskeletal Manifestations in Acute Lymphoblastic Leukemia: A Decade's Experience from a Referral Center in Southern India.
Indian pediatricsA child with systemic onset juvenile idiopathic arthritis and nephrotic syndrome.
Pediatric nephrology (Berlin, Germany)Diagnostic guidelines for familial hemophagocytic lymphohistiocytosis revisited.
BloodFunctional status of Indian children with juvenile idiopathic arthritis.
Rheumatology and immunology researchMacrophage activation syndrome in a Sudanese child: a case report from Sudan.
Annals of medicine and surgery (2012)Case report: Success of allogeneic hematopoietic stem cell transplantation for refractory systemic-onset juvenile idiopathic arthritis.
Frontiers in medicineDisease evolution in systemic juvenile idiopathic arthritis: an international, observational cohort study through JIRcohort.
Pediatric rheumatology online journal[Refractory macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis treated with canakinumab].
Zhonghua er ke za zhi = Chinese journal of pediatricsUse of Intravenous Anakinra for Management of Pediatric Cytokine Storm Syndromes at an Academic Medical Center.
Hospital pharmacyIFIH1 and DDX58 gene variants in pediatric rheumatic diseases.
World journal of clinical pediatricsTocilizumab-induced hypofibrinogenemia in patients with systemic-onset juvenile idiopathic arthritis.
Scientific reportsRescue of Pap-Mas in Systemic JIA Using Janus Kinase Inhibitors, Case Report and Systematic Review.
Journal of clinical medicineADA2 deficiency (DADA2) misdiagnosed as systemic onset juvenile idiopathic arthritis in a child carrying a novel compound heterozygous ADA2 mutation: a case report.
Translational pediatricsEfficacy of anakinra treatment in pediatric rheumatic diseases: Our single-center experience.
Archives of rheumatologyTherapeutic plasma exchange in refractory macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a case-based review.
Rheumatology internationalJAK inhibitors in difficult-to-treat adult-onset Still's disease and systemic-onset juvenile idiopathic arthritis.
Rheumatology (Oxford, England)Secondary hemophagocytic lymphohistiocytosis associated with Rocky Mountain spotted fever in a toddler: A case report.
EJHaemRefractory systemic onset juvenile idiopathic arthritis: current challenges and future perspectives.
Annals of medicineUrticaria and angioedema in children and adolescents: diagnostic challenge.
Allergologia et immunopathologiaAdult-onset and Juvenile-onset Still's Disease: A Comparative Study of Both Sides.
Current rheumatology reviewsCOVID-19 infection among patients with autoinflammatory diseases: a study on 117 French patients compared with 1545 from the French RMD COVID-19 cohort: COVIMAI - the French cohort study of SARS-CoV-2 infection in patient with systemic autoinflammatory diseases.
RMD openSevere Immediate and Delayed Hypersensitivity Reactions to Biologics in a Toddler With Systemic Juvenile Idiopathic Arthritis.
Journal of investigative medicine high impact case reportsA case of multisystem inflammatory syndrome in children presenting as systemic onset juvenile idiopathic arthritis.
JPMA. The Journal of the Pakistan Medical Association[Recommendations of diagnosis and treatment of juvenile idiopathic arthritis in China].
Zhonghua nei ke za zhiDetection and Prediction of Macrophage Activation Syndrome in Still's Disease.
Journal of clinical medicineCrohn's disease in a patient with systemic onset juvenile idiopathic arthritis. Association or associated side effect of treatment?
Reumatologia clinicaStill's Disease Mortality Trends in France, 1979-2016: A Multiple-Cause-of-Death Study.
Journal of clinical medicineAdult-onset Still's disease or systemic-onset juvenile idiopathic arthritis and spondyloarthritis: overlapping syndrome or phenotype shift?
Rheumatology (Oxford, England)Visceral Toxocariasis Presenting as Systemic-Onset Juvenile Idiopathic Arthritis.
Journal of tropical pediatricsOverlapping Features in Kawasaki Disease-Related Arthritis and Systemic-Onset Juvenile Idiopathic Arthritis: A Nationwide Study in Japan.
Frontiers in pediatricsDiffuse alveolar hemorrhage in children with trisomy 21.
Pediatric rheumatology online journalThe immunoregulatory function of peripheral blood CD71+ erythroid cells in systemic-onset juvenile idiopathic arthritis.
Scientific reportsClinical Phenotypes of Adult-Onset Still's Disease: New Insights from Pathophysiology and Literature Findings.
Journal of clinical medicineBrucellosis as a cause of hyperferritinemia in systemic arthritis.
Pediatrics international : official journal of the Japan Pediatric SocietyThe Association of Serum IL-10 Levels with the Disease Activity in Systemic-Onset Juvenile Idiopathic Arthritis Patients.
Mediators of inflammationAdult-Onset Still's Disease: Clinical Aspects and Therapeutic Approach.
Journal of clinical medicineCase Report: Life-Threatening Macrophage Activation Syndrome With Fulminant Myocarditis Successfully Rescued by High Dose Intravenous Anakinra.
Frontiers in pediatricsPerimyocarditis as first sign of systemic onset juvenile idiopathic arthritis treated successfully with anakinra: a case-based review.
Acta bio-medica : Atenei ParmensisOutcome of PatientsWith Systemic Onset Juvenile Idiopathic Arthritis With Macrophage Activation Syndrome at Onset.
Indian pediatricsMacrophage activation syndrome in systemic lupus erythematosus and systemic-onset juvenile idiopathic arthritis: a retrospective study of similarities and dissimilarities.
Rheumatology internationalKawasaki disease mimickers.
Pediatrics international : official journal of the Japan Pediatric SocietyPulmonary Manifestations of Rheumatic Diseases in Children.
Pediatric clinics of North AmericaSafety and efficacy of anakinra as first-line or second-line therapy for systemic onset juvenile idiopathic arthritis - data from the German BIKER registry.
Expert opinion on drug safetyHemophagocytic Lymphohistiocytosis in a PICU of a Developing Economy: Clinical Profile, Intensive Care Needs, Outcome, and Predictors of Mortality.
Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care SocietiesAiming high: quantifying inflammation in systemic onset juvenile idiopathic arthritis (sJIA), a multi-faceted and complex inflammatory disease.
Rheumatology (Oxford, England)Communicating Calf Swelling in Systemic-Onset Juvenile Idiopathic Arthritis.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesRheumatologists' perspective on coronavirus disease 19 (COVID-19) and potential therapeutic targets.
Clinical rheumatologySevere Recurrent Fever Episodes With Clinical Diagnosis of Hemophagocytic Lymphohistiocytosis, Incomplete Kawasaki Disease and Systemic-Onset Juvenile Idiopathic Arthritis: A Case Report and Literature Review.
Frontiers in pediatricsComparison of Early vs. Delayed Anakinra Treatment in Patients With Adult Onset Still's Disease and Effect on Clinical and Laboratory Outcomes.
Frontiers in medicineAutoinflammatory diseases in childhood, part 2: polygenic syndromes.
Pediatric radiologyAutoinflammatory diseases in childhood, part 1: monogenic syndromes.
Pediatric radiologyLong-term surveillance of biologic therapies in systemic-onset juvenile idiopathic arthritis: data from the German BIKER registry.
Rheumatology (Oxford, England)Population pharmacokinetics of tacrolimus in pediatric patients with systemic-onset juvenile idiopathic arthritis: Initial dosage recommendations.
Experimental and therapeutic medicineCharacterization of microbiota in systemic-onset juvenile idiopathic arthritis with different disease severities.
World journal of clinical casesExtreme thrombocytosis in systemic juvenile idiopathic arthritis. A case report.
Italian journal of pediatricsCyclosporine for Systemic Onset Juvenile Idiopathic Arthritis: Current Stand and Future Directions.
Indian journal of pediatricsAchromobacter xylosoxidans Sepsis Unveiling X-linked Agammaglobulinemia Masquerading as Systemic-onset Juvenile Idiopathic Arthritis.
Indian pediatricsLong-Term Retention Rate of Anakinra in Adult Onset Still's Disease and Predictive Factors for Treatment Response.
Frontiers in pharmacologyComparative study of Interleukin-18 (IL-18) serum levels in adult onset Still's disease (AOSD) and systemic onset juvenile idiopathic arthritis (sJIA) and its use as a biomarker for diagnosis and evaluation of disease activity.
BMC rheumatologyTreatment of patients with systemic-onset juvenile idiopathic arthritis with tacrolimus.
Experimental and therapeutic medicinePredictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis.
The Journal of rheumatologyThe role of extracellular histones in systemic-onset juvenile idiopathic arthritis.
Italian journal of pediatricsSystemic onset juvenile idiopathic arthritis: a single center experience.
The Turkish journal of pediatricsTocilizumab in the treatment of systemic-onset juvenile idiopathic arthritis - single-centre experience.
ReumatologiaMethotrexate-Induced Vasculitis in a Child with Systemic Onset Juvenile Idiopathic Arthritis.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesNovel presentations of periodic fever syndromes: Discrepancies between genetic and clinical diagnoses.
European journal of rheumatologyImpact of biologics on disease course in systemic onset juvenile idiopathic arthritis.
Clinical rheumatologyMolecular genetic analysis for periodic fever syndromes: a supplemental role for the diagnosis of adult-onset Still's disease.
Clinical rheumatologyTocilizumab in Giant Cell Arteritis.
Cardiology in review[Recognition of systemic-onset juvenile idiopathic arthritis at the genetic level].
Zhonghua er ke za zhi = Chinese journal of pediatricsRemission of Refractory Systemic-Onset Juvenile Idiopathic Arthritis After Treatment With Siltuximab.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases[Systemic juvenile onset idiopathic arthritis and adult onset still disease].
Revue medicale suisseReversal of Sepsis-Like Features of Neutrophils by Interleukin-1 Blockade in Patients With Systemic-Onset Juvenile Idiopathic Arthritis.
Arthritis & rheumatology (Hoboken, N.J.)Reasons for inactive disease and flare in systemic onset juvenile idiopathic arthritis patients during tocilizumab treatment.
Clinical and experimental rheumatologyProteinuria in children with juvenile idiopathic arthritis: Making the case for early urinary screening.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaExperience with etanercept, tocilizumab and interleukin-1 inhibitors in systemic onset juvenile idiopathic arthritis patients from the BIKER registry.
Arthritis research & therapySystemic-onset juvenile idiopathic arthritis and incomplete Kawasaki disease may belong to a single clinical syndrome within a spectrum of severity.
Clinical and experimental rheumatologyClinical improvement of renal amyloidosis in a patient with systemic-onset juvenile idiopathic arthritis who received tocilizumab treatment: a case report and literature review.
BMC nephrologyA Systemic-Onset Juvenile Idiopathic Arthritis Patient with Reduced Anakinra Treatment Admitted with an Attack.
The Eurasian journal of medicineProinflammatory Cytokine Environments Can Drive Interleukin-17 Overexpression by γ/δ T Cells in Systemic Juvenile Idiopathic Arthritis.
Arthritis & rheumatology (Hoboken, N.J.)The novel use of combined IL-1 and IL-6 inhibition in a patient with severe, aggressive, erosive, systemic-onset juvenile idiopathic arthritis.
European journal of rheumatologyProtecting Bone Health in Pediatric Rheumatic Diseases: Pharmacological Considerations.
Paediatric drugsIn-hospital mortality in febrile lupus patients based on 2016 EULAR/ACR/PRINTO classification criteria for macrophage activation syndrome.
Seminars in arthritis and rheumatismCanakinumab for first line steroid-free treatment in a child with systemic-onset juvenile idiopathic arthritis.
Scandinavian journal of rheumatologyCourse, Outcome and Complications in Children with Systemic Onset Juvenile Idiopathic Arthritis.
Indian journal of pediatricsExtremely elevated IL-18 levels may help distinguish systemic-onset juvenile idiopathic arthritis from other febrile diseases.
Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas[Correctly adDRESS the cause of hemophagocytic lymphohistiocytosis].
Archives de pediatrie : organe officiel de la Societe francaise de pediatrieSystemic-onset juvenile idiopathic arthritis or incomplete Kawasaki disease: a diagnostic challenge.
Clinical and experimental rheumatologySystemic onset juvenile idiopathic arthritis and exposure to fine particulate air pollution.
Clinical and experimental rheumatologyOperative stabilization of the remaining mobile segment in ankylosed cervical spine in systemic onset - juvenile idiopathic arthritis: A case report.
World journal of orthopedicsSystemic onset juvenile idiopathic arthritis with macrophage activation syndrome and coronary artery dilatation misdiagnosed as Kawasaki disease.
The Turkish journal of pediatricsMacrophage activation syndrome in a patient with systemic onset of the juvenile idiopathic arthritis.
ReumatologiaSystemic-onset juvenile idiopathic arthritis.
Autoimmunity reviewsAssessment of vascular function in systemic onset juvenile idiopathic arthritis.
Clinical rheumatologyAtypical Cutaneous Manifestations in Adult Onset Still's Disease.
Case reports in rheumatology[Juvenile idiopathic arthritis: Definition and classification].
Archives de pediatrie : organe officiel de la Societe francaise de pediatrieShould 2016 Criteria for Macrophage Activation Syndrome be applied in children with Kawasaki disease, as well as with systemic-onset juvenile idiopathic arthritis?
Annals of the rheumatic diseasesBullous Erythema Nodosum Leprosum masquerading as systemic onset juvenile idiopathic arthritis: A case report.
Leprosy reviewPotential Effects of Interleukins on the Pathogenesis of Systemic Onset Juvenile Idiopathic Arthritis.
Pediatrics and neonatologyIL-6 blockers in systemic onset juvenile idiopathic arthritis.
ImmunotherapyA safety evaluation of canakinumab for the treatment of systemic onset juvenile idiopathic arthritis.
Expert opinion on drug safetySuccessful treatment of macrophage activation syndrome due to systemic onset juvenile idiopathic arthritis with antithymocyte globulin.
Rheumatology internationalNewly recognized Mendelian disorders with rheumatic manifestations.
Current opinion in rheumatologyFrom bench to bedside and back again: translational research in autoinflammation.
Nature reviews. RheumatologyDiagnosis of systemic-onset juvenile idiopathic arthritis after treatment for presumed Kawasaki disease.
The Journal of pediatricsEffect of etanercept on refractory systemic-onset juvenile idiopathic arthritis.
World journal of pediatrics : WJPTreatment of systemic-onset juvenile arthritis with canakinumab.
Open access rheumatology : research and reviewsSuccessful treatment with tocilizumab every 4 weeks of a low disease activity group who achieve a drug-free remission in patients with systemic-onset juvenile idiopathic arthritis.
Pediatric rheumatology online journalMyositis as a presenting feature of systemic onset juvenile idiopathic arthritis.
Journal of paediatrics and child healthAdult-onset Still's disease-pathogenesis, clinical manifestations, and new treatment options.
Annals of medicine[Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis].
Revista brasileira de reumatologiaAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Artrite juvenil idiopática de início sistêmico.
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Artrite juvenil idiopática de início sistêmico
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Systemic Onset Juvenile Idiopathic Arthritis in Sickle Cell Anaemia: A Diagnostic Odyssey.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion· 2026· PMID 41522543mais citado
- An abnormal immune response masquerading as infectious conditions.
- Rescue Therapy With Continuous Intravenous Anakinra Infusion and Plasma Exchange in Refractory Cytokine Storm of Systemic JIA: A Case Report.
- Tofacitinib as an adjuvant treatment for pediatric Still's disease.
- Acute lymphoblastic leukaemia presenting as chronic recurrent multifocal osteomyelitis.
- Effectiveness of Canakinumab for First-Line Steroid-Free Treatment in Systemic-Onset Juvenile Idiopathic Arthritis and Juvenile Still Disease.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:85414(Orphanet)
- MONDO:0019434(MONDO)
- GARD:10966(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q17148432(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
