BACKGROUND Systemic-onset juvenile idiopathic arthritis (SoJIA) is one of most serious subtypes of juvenile idiopathic arthritis. Although the pathogenesis of SoJIA remains unclear, several studies have suggested a co...BACKGROUND Systemic-onset juvenile idiopathic arthritis (SoJIA) is one of most serious subtypes of juvenile idiopathic arthritis. Although the pathogenesis of SoJIA remains unclear, several studies have suggested a correlation between gut dysbiosis and JIA. Further understanding of the intestinal microbiome may help to establish alternative ways to treat, or even prevent, the disease. AIM To explore alterations in fecal microbiota profiles in SoJIA patients and to evaluate the correlations between microbiota and clinical parameters. METHODS We conducted an observational single-center study at the Pediatric Department of Peking Union Medical College Hospital. Children who were diagnosed with SoJIA at our institution and followed for a minimum period of six months after diagnosis were recruited for the study. Healthy children were recruited as a control group (HS group) during the same period. Clinical data and stool samples were collected from SoJIA patients when they visited the hospital. RESULTS The SoJIA group included 17 active and 15 inactive consecutively recruited children;the control group consisted of 32 children. Firmicutes and Bacteroidetes were the two most abundant phyla among the total sample of SoJIA children and controls. There was a significant difference among the three groups in observed species, which was the highest in the Active-SoJIA group, followed by the Inactive-SoJIA group and then HS group (Active-SoJIA vs HS: P = 0.000;and Inactive-SoJIA vs HS: P = 0.005). We observed a lower Firmicutes/Bacteroidetes ratio in SoJIA patients (3.28 ± 4.47 in Active-SoJIA, 5.36 ± 8.39 in Inactive-SoJIA,and 5.67 ± 3.92 in HS). We also observed decreased abundances of Ruminococcaceae (14.9% in Active-SoJIA, 17.3% in Inactive-SoJIA, and 22.8% in HS;Active-SoJIA vs HS: P = 0.005) and Faecalibacterium (5.1% in Active-SoJIA, 9.9% in Inactive-SoJIA, and 13.0% in HS;Active-SoJIA vs HS: P = 0.000) in SoJIA compared with HS. By contrast, the abundance of Bacteroidaceae was the highest in the Active-SoJIA group, followed by the Inactive-SoJIA and HS groups (16.5% in Active-SoJIA, 12.8% in Inactive-SoJIA, and 9.7% in HS;Active-SoJIA vs HS: P = 0.03). The Spearman correlation analysis revealed a negative correlation between Proteobacteria or Enterobacteriaceae and juvenile arthritis disease activity score on 27 joints (JADAS-27). CONCLUSION The composition of the intestinal microbiota is different in SoJIA patients compared with healthy children. The dysbiosis presents partial restoration in inactive status patients.展开更多
Background and Objective: Systemic-onset juvenile idiopathic arthritis (JIA) is a major and prevalent subset of arthritis among children and it has a broad spectrum of clinical presentation, course and prognosis. This...Background and Objective: Systemic-onset juvenile idiopathic arthritis (JIA) is a major and prevalent subset of arthritis among children and it has a broad spectrum of clinical presentation, course and prognosis. This study described the clinical presentation of systemic-onset JIA in a Saudi-based cohort. Methods: A retrospective chart review was performed of the medical records of children with systemic-onset JIA who were followed up at King Abdul Aziz University Hospital, Jeddah, between January 1997 and December 2013. Patients’ files were reviewed for demographic, clinical, and paraclinical data, which were analyzed using the statistical Package for the Social Sciences. Results: We included 20 patients of both genders (8 boys and 12 girls). The mean age of disease onset was 7 (4.5) years. The most common presenting symptoms were fever (100%), arthritis (100%), and rash (55%). Hepatomegaly (5%), abdominal (5%) and pulmonary manifestations (3%) were less frequent manifestations. Most patients had high white blood cell counts (50%), elevated erythrocyte sedimentation rates (80%) and C-reactive protein levels (90%). The interval between onset of symptoms and diagnosis was 9.4 (12.5) weeks. Patients were treated with non-steroidal anti-inflammatory drugs, methotrexate, steroids, anti-tumor necrosis agents, and disease-modifying anti-rheumatic drugs. Bone marrow biopsy was conducted to exclude malignancy in 20% of the patients. Conclusion: Saudi children with systemic-onset JIA present with prolonged fever and arthritis (mainly oligoarticular rather than polyarticular). Physicians should be aware of the presentation of systemic-onset JIA in our setting in order to make prompt diagnosis and treatment decisions as early as possible. Carful follow-up of febrile patients is paramount to reaching the diagnosis early and initiating treatment.展开更多
Objective: To describe the epidemiological, clinical, paraclinical and therapeutic aspects of systemic juvenile idiopathic arthritis observed in Abidjan. Materials and Method: This retrospective and descriptive study ...Objective: To describe the epidemiological, clinical, paraclinical and therapeutic aspects of systemic juvenile idiopathic arthritis observed in Abidjan. Materials and Method: This retrospective and descriptive study covered 13 children suffering from systemic juvenile idiopathic arthritis selected in the Rheumatology Department of University Hospital Center of Cocody in Abidjan (Cote d’Ivoire) from January 2005 to December 2015. We were interested to the sociodemographical, clinical, paraclinical and therapeutic aspects. Results: The systemic form of the juvenile idiopathic arthritis represented 0.2% of the 4608 rheumatologic diseases and 70.58% of the JIA. We selected 6 boys and 7 girls, with an average age of 10.8 years and mostly going to school (84.61%). The diagnostic delay was 18 months. The main clinical signs were fever and joint damage observed each in 100% of cases, impaired general condition (92.30%) and tumor syndrome (83.33%). Biological signs were characterized by hyperleukocytosis (69.20%) and the presence of a biologic inflammatory syndrome (on average, erythrocyte sedimentation rate 59.6 mm and C Reactive Protein 56.4 mg/l). The cervical damage was the essential functional complication (38.46%). The major treatment has been a therapeutic combination based on corticotherapy and methotrexate (100%) with 1 death case by macrophage activation syndrome. Conclusion: Systemic juvenile idiopathic arthritis is rarely diagnosed in the rheumatologic practice in Abidjan. It concerns children relatively big, and is characterized by a febrile polyarthritis with impaired general condition and tumor syndrome. This systemic form is treated by corticotherapy and methotrexate.展开更多
Systemic </span><span><span style="font-family:Verdana;">juvenile idiopathic arthritis is classified as an autoimmune entity and a subtype of juvenile idiopathic arthritis, although it has ...Systemic </span><span><span style="font-family:Verdana;">juvenile idiopathic arthritis is classified as an autoimmune entity and a subtype of juvenile idiopathic arthritis, although it has many features of autoinflammatory-type of diseases. This review article will elaborate on the disease’s pathogenesis and its proposed relation to autoinflammatory diseases including defective innate immunity and phagocytosis response leading </span><span style="font-family:Verdana;">excessive</span><span style="font-family:Verdana;"> cytokine release. It also explains the disease’s epidemiology, clinical phenotype, diagnostic challenges, complications </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> current advancements in the treatment of systemic juvenile idiopathic arthritis, such as IL-1 and IL-6 antagonists </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> their impact on the disease trajectory. Care of patients with systemic juvenile idiopathic arthritis requires a comprehensive multidisciplinary team to optimize the care and avoid complications of the disease itself such as growth impairment, macrophage activation syndrome </span><span style="font-family:Verdana;">or</span><span style="font-family:Verdana;"> the complications of immunosuppressant and immun</span></span><span style="font-family:Verdana;">omodulatory treatments.展开更多
文摘BACKGROUND Systemic-onset juvenile idiopathic arthritis (SoJIA) is one of most serious subtypes of juvenile idiopathic arthritis. Although the pathogenesis of SoJIA remains unclear, several studies have suggested a correlation between gut dysbiosis and JIA. Further understanding of the intestinal microbiome may help to establish alternative ways to treat, or even prevent, the disease. AIM To explore alterations in fecal microbiota profiles in SoJIA patients and to evaluate the correlations between microbiota and clinical parameters. METHODS We conducted an observational single-center study at the Pediatric Department of Peking Union Medical College Hospital. Children who were diagnosed with SoJIA at our institution and followed for a minimum period of six months after diagnosis were recruited for the study. Healthy children were recruited as a control group (HS group) during the same period. Clinical data and stool samples were collected from SoJIA patients when they visited the hospital. RESULTS The SoJIA group included 17 active and 15 inactive consecutively recruited children;the control group consisted of 32 children. Firmicutes and Bacteroidetes were the two most abundant phyla among the total sample of SoJIA children and controls. There was a significant difference among the three groups in observed species, which was the highest in the Active-SoJIA group, followed by the Inactive-SoJIA group and then HS group (Active-SoJIA vs HS: P = 0.000;and Inactive-SoJIA vs HS: P = 0.005). We observed a lower Firmicutes/Bacteroidetes ratio in SoJIA patients (3.28 ± 4.47 in Active-SoJIA, 5.36 ± 8.39 in Inactive-SoJIA,and 5.67 ± 3.92 in HS). We also observed decreased abundances of Ruminococcaceae (14.9% in Active-SoJIA, 17.3% in Inactive-SoJIA, and 22.8% in HS;Active-SoJIA vs HS: P = 0.005) and Faecalibacterium (5.1% in Active-SoJIA, 9.9% in Inactive-SoJIA, and 13.0% in HS;Active-SoJIA vs HS: P = 0.000) in SoJIA compared with HS. By contrast, the abundance of Bacteroidaceae was the highest in the Active-SoJIA group, followed by the Inactive-SoJIA and HS groups (16.5% in Active-SoJIA, 12.8% in Inactive-SoJIA, and 9.7% in HS;Active-SoJIA vs HS: P = 0.03). The Spearman correlation analysis revealed a negative correlation between Proteobacteria or Enterobacteriaceae and juvenile arthritis disease activity score on 27 joints (JADAS-27). CONCLUSION The composition of the intestinal microbiota is different in SoJIA patients compared with healthy children. The dysbiosis presents partial restoration in inactive status patients.
文摘Background and Objective: Systemic-onset juvenile idiopathic arthritis (JIA) is a major and prevalent subset of arthritis among children and it has a broad spectrum of clinical presentation, course and prognosis. This study described the clinical presentation of systemic-onset JIA in a Saudi-based cohort. Methods: A retrospective chart review was performed of the medical records of children with systemic-onset JIA who were followed up at King Abdul Aziz University Hospital, Jeddah, between January 1997 and December 2013. Patients’ files were reviewed for demographic, clinical, and paraclinical data, which were analyzed using the statistical Package for the Social Sciences. Results: We included 20 patients of both genders (8 boys and 12 girls). The mean age of disease onset was 7 (4.5) years. The most common presenting symptoms were fever (100%), arthritis (100%), and rash (55%). Hepatomegaly (5%), abdominal (5%) and pulmonary manifestations (3%) were less frequent manifestations. Most patients had high white blood cell counts (50%), elevated erythrocyte sedimentation rates (80%) and C-reactive protein levels (90%). The interval between onset of symptoms and diagnosis was 9.4 (12.5) weeks. Patients were treated with non-steroidal anti-inflammatory drugs, methotrexate, steroids, anti-tumor necrosis agents, and disease-modifying anti-rheumatic drugs. Bone marrow biopsy was conducted to exclude malignancy in 20% of the patients. Conclusion: Saudi children with systemic-onset JIA present with prolonged fever and arthritis (mainly oligoarticular rather than polyarticular). Physicians should be aware of the presentation of systemic-onset JIA in our setting in order to make prompt diagnosis and treatment decisions as early as possible. Carful follow-up of febrile patients is paramount to reaching the diagnosis early and initiating treatment.
文摘Objective: To describe the epidemiological, clinical, paraclinical and therapeutic aspects of systemic juvenile idiopathic arthritis observed in Abidjan. Materials and Method: This retrospective and descriptive study covered 13 children suffering from systemic juvenile idiopathic arthritis selected in the Rheumatology Department of University Hospital Center of Cocody in Abidjan (Cote d’Ivoire) from January 2005 to December 2015. We were interested to the sociodemographical, clinical, paraclinical and therapeutic aspects. Results: The systemic form of the juvenile idiopathic arthritis represented 0.2% of the 4608 rheumatologic diseases and 70.58% of the JIA. We selected 6 boys and 7 girls, with an average age of 10.8 years and mostly going to school (84.61%). The diagnostic delay was 18 months. The main clinical signs were fever and joint damage observed each in 100% of cases, impaired general condition (92.30%) and tumor syndrome (83.33%). Biological signs were characterized by hyperleukocytosis (69.20%) and the presence of a biologic inflammatory syndrome (on average, erythrocyte sedimentation rate 59.6 mm and C Reactive Protein 56.4 mg/l). The cervical damage was the essential functional complication (38.46%). The major treatment has been a therapeutic combination based on corticotherapy and methotrexate (100%) with 1 death case by macrophage activation syndrome. Conclusion: Systemic juvenile idiopathic arthritis is rarely diagnosed in the rheumatologic practice in Abidjan. It concerns children relatively big, and is characterized by a febrile polyarthritis with impaired general condition and tumor syndrome. This systemic form is treated by corticotherapy and methotrexate.
文摘Systemic </span><span><span style="font-family:Verdana;">juvenile idiopathic arthritis is classified as an autoimmune entity and a subtype of juvenile idiopathic arthritis, although it has many features of autoinflammatory-type of diseases. This review article will elaborate on the disease’s pathogenesis and its proposed relation to autoinflammatory diseases including defective innate immunity and phagocytosis response leading </span><span style="font-family:Verdana;">excessive</span><span style="font-family:Verdana;"> cytokine release. It also explains the disease’s epidemiology, clinical phenotype, diagnostic challenges, complications </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> current advancements in the treatment of systemic juvenile idiopathic arthritis, such as IL-1 and IL-6 antagonists </span><span style="font-family:Verdana;">and</span><span style="font-family:Verdana;"> their impact on the disease trajectory. Care of patients with systemic juvenile idiopathic arthritis requires a comprehensive multidisciplinary team to optimize the care and avoid complications of the disease itself such as growth impairment, macrophage activation syndrome </span><span style="font-family:Verdana;">or</span><span style="font-family:Verdana;"> the complications of immunosuppressant and immun</span></span><span style="font-family:Verdana;">omodulatory treatments.
