Systemic-onset JRA is characterized by spiking fevers lasting more than two weeks, typically occurring once, twice each day, with temperature returning to the normal or below normal. There are no specific diagnostic t...Systemic-onset JRA is characterized by spiking fevers lasting more than two weeks, typically occurring once, twice each day, with temperature returning to the normal or below normal. There are no specific diagnostic tests for systemic JRA. High level of serum ferritin is the most important, no specific, diagnostic finding associated.展开更多
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: Juvenile Rheumatoid Arthritis (JRA) is an inflammatory disease that affects the joints of children and is associated with ocular complications, like uveitis and cataract. Patients with such complications c...Background: Juvenile Rheumatoid Arthritis (JRA) is an inflammatory disease that affects the joints of children and is associated with ocular complications, like uveitis and cataract. Patients with such complications can benefit from a low vision assessment to improve their functionality and their quality of life. In this case study, the importance of early detection and management of ocular complications of JRA, as well as visual rehabilitation through a low vision assessment, are highlighted and discussed. Case Presentation: A 12-year-old female of East Indian descent presented to the Low Vision Center of the University of the West Indies Optometry Eye Unit, bilaterally aphakic (absence of crystalline lens) as a result of complications she developed secondary to a systemic condition diagnosed as JRA. She presented with a distance visual acuity of 2.30 logMAR in the RE, and 1.64 logMAR in the LE, at near she was able to read 8.0 M and 3.2 M at a distance of 8 cm for RE & LE respectively. She has a history of ocular disorder secondary to JRA such as cataract, uveitis, upon low vision assessment a new spectacle prescription and an illuminated stand magnifier of 8D was issued, the patient was satisfied with the outcome of the treatment, management and low vision device issued. Conclusion: At the end of the low vision assessment, the patient was extremely satisfied and looked forward to the prospect of returning to school. Although JRA can have severe ocular complications, with the correct management, a patient’s quality of life can be dramatically improved thus stressing the importance of these types of visual assessments.展开更多
Objectives: To evaluate the effect of Juvenile Idiopathic Rheumatoid Ar-thritis (JIA) on the health-related quality of life (HRQOL) in Saudi children. Methods: A cross-sectional study was conducted in a tertiary hospi...Objectives: To evaluate the effect of Juvenile Idiopathic Rheumatoid Ar-thritis (JIA) on the health-related quality of life (HRQOL) in Saudi children. Methods: A cross-sectional study was conducted in a tertiary hospital in Jeddah, Saudi Arabia to evaluate the HRQOL of children aged ≤ 18 years who had JIA using the childhood health assessment questionnaire modified for Arab children (CHAQ-MAC). Such questionnaire investigates 34 activities of daily life (ADL) classified into 8 life domains. Children or their parents were invited for face-to-face interview, and a phone interview was done for patients who missed their appointments during the period between February and July 2017. A statistical model was used to calculate a total CHAQ.MAC score (range = 0 - 33;Cronbach’s alpha = 0.966);with higher values indicating poorer HRQOL. Results: Of a total of 44 children (male ratio = 0.63;mean ± SD age = 9.95 ± 5.44), Systemic-onset JIA was the most frequent type (27.3%), followed by polyarticular (15.9%) and oligoarticular (13.6%). Pain was reported among 43.2% (frequently in the knee, in 27.3%) whereas morning stiffness was reported in 20.5%. The mean CHAQ.MAC score = 2.89 (75th centile = 3.00). With respect of ADLs, up to 22.7% of the children complained of difficulty;and 31.8% reported a difficulty in at least one of the 34 investigated ADLs. With respect of the life domain, children reported difficulties for activities (27.3%), dressing & grooming and hygiene (13.6%), and eating (6.82%). According to the life domain, 4.5% to 13.6% of the children needed help to execute the related ADLs and up to 9.1% used aids or devices. Poor HRQOL was associated with articular pain (p = 0.003) and specific medication (p = 0.043). Con-clusion: Children with arthralgia and those on specific treatment are at higher risk of impaired QOL, which emphasizes the need for systematic screening for treatment adverse effects and joint pain and implementation of efficient management to improve HRQOL.展开更多
We herewith report the rare case of a patient with juvenile rheumatoid arthritis who developed on 2 occasions the clinical picture of an isolated unilateral palsy of the inferior oblique muscle—Brown’s syndrome, fol...We herewith report the rare case of a patient with juvenile rheumatoid arthritis who developed on 2 occasions the clinical picture of an isolated unilateral palsy of the inferior oblique muscle—Brown’s syndrome, following pregnancies, each time in a different eye. Although the eye is frequently involved in rheumatoid arthritis (RA), the Brown’s syndrome is seldom reported in literature with regards to RA.展开更多
Background:Treatment of systemic-onset juvenile idiopathic arthritis(So-JIA)is challenging,and the effi cacy of injectable recombinant human tumor necrosis factor type 1 receptor-antibody fusion protein(etanercept)on ...Background:Treatment of systemic-onset juvenile idiopathic arthritis(So-JIA)is challenging,and the effi cacy of injectable recombinant human tumor necrosis factor type 1 receptor-antibody fusion protein(etanercept)on So-JIA has been controversial.Methods:We retrospectively studied 12 patients with refractory systemic juvenile arthritis treated with etanercept at our hospital in the past 5 years.The 12 patients were divided into a corticosteroid-dependent group(n=7)and an ineffective group(n=5)on the basis of their responses to treatment before the administration of etanercept.Etanercept was added to the treatment without substantially changing the original regimens in general,and doses,and signs of efficacy including alleviation or resolution of symptoms such as high fever,infl ammatory arthropathy,eruption rash,hydrohymenitis,as well as changes in the levels of laboratory infl ammatory markers such as the white blood cell count,erythrocyte sedimentation rate,levels of C-reactive protein and serum ferritin were recorded.Results:Etanercept was withdrawn after the first dose from one patient in the corticosteroid-dependent group because of a systemic allergic rash,and was also withdrawn from one patient in the ineffective group after 2 months of treatment owing to ineffi cacy;the remaining 10 patients completed the entire treatment protocol,at which point etanercept was discontinued.At that time,clinical symptoms and laboratory infl ammatory markers of the remaining patients were within the normal range and the mean dose of prednisone was 0.18 mg/kg per day,an 81%decrease from the mean dose at baseline.At present,the corticosteroid has been discontinued and only methotrexate maintenance treatment is used in 3 patients;the other 7 patients are treated with prednisone and methotrexate maintenance therapy.All of the 10 patients are in a medicated remission with no recurrence.Conclusions:In the treatment of patients with refractory So-JIA,the principles of individual therapy and combinations of drugs should be followed.Etanercept is an important and valid candidate for use in such combined treatment strategies.展开更多
文摘Systemic-onset JRA is characterized by spiking fevers lasting more than two weeks, typically occurring once, twice each day, with temperature returning to the normal or below normal. There are no specific diagnostic tests for systemic JRA. High level of serum ferritin is the most important, no specific, diagnostic finding associated.
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: Juvenile Rheumatoid Arthritis (JRA) is an inflammatory disease that affects the joints of children and is associated with ocular complications, like uveitis and cataract. Patients with such complications can benefit from a low vision assessment to improve their functionality and their quality of life. In this case study, the importance of early detection and management of ocular complications of JRA, as well as visual rehabilitation through a low vision assessment, are highlighted and discussed. Case Presentation: A 12-year-old female of East Indian descent presented to the Low Vision Center of the University of the West Indies Optometry Eye Unit, bilaterally aphakic (absence of crystalline lens) as a result of complications she developed secondary to a systemic condition diagnosed as JRA. She presented with a distance visual acuity of 2.30 logMAR in the RE, and 1.64 logMAR in the LE, at near she was able to read 8.0 M and 3.2 M at a distance of 8 cm for RE & LE respectively. She has a history of ocular disorder secondary to JRA such as cataract, uveitis, upon low vision assessment a new spectacle prescription and an illuminated stand magnifier of 8D was issued, the patient was satisfied with the outcome of the treatment, management and low vision device issued. Conclusion: At the end of the low vision assessment, the patient was extremely satisfied and looked forward to the prospect of returning to school. Although JRA can have severe ocular complications, with the correct management, a patient’s quality of life can be dramatically improved thus stressing the importance of these types of visual assessments.
文摘Objectives: To evaluate the effect of Juvenile Idiopathic Rheumatoid Ar-thritis (JIA) on the health-related quality of life (HRQOL) in Saudi children. Methods: A cross-sectional study was conducted in a tertiary hospital in Jeddah, Saudi Arabia to evaluate the HRQOL of children aged ≤ 18 years who had JIA using the childhood health assessment questionnaire modified for Arab children (CHAQ-MAC). Such questionnaire investigates 34 activities of daily life (ADL) classified into 8 life domains. Children or their parents were invited for face-to-face interview, and a phone interview was done for patients who missed their appointments during the period between February and July 2017. A statistical model was used to calculate a total CHAQ.MAC score (range = 0 - 33;Cronbach’s alpha = 0.966);with higher values indicating poorer HRQOL. Results: Of a total of 44 children (male ratio = 0.63;mean ± SD age = 9.95 ± 5.44), Systemic-onset JIA was the most frequent type (27.3%), followed by polyarticular (15.9%) and oligoarticular (13.6%). Pain was reported among 43.2% (frequently in the knee, in 27.3%) whereas morning stiffness was reported in 20.5%. The mean CHAQ.MAC score = 2.89 (75th centile = 3.00). With respect of ADLs, up to 22.7% of the children complained of difficulty;and 31.8% reported a difficulty in at least one of the 34 investigated ADLs. With respect of the life domain, children reported difficulties for activities (27.3%), dressing & grooming and hygiene (13.6%), and eating (6.82%). According to the life domain, 4.5% to 13.6% of the children needed help to execute the related ADLs and up to 9.1% used aids or devices. Poor HRQOL was associated with articular pain (p = 0.003) and specific medication (p = 0.043). Con-clusion: Children with arthralgia and those on specific treatment are at higher risk of impaired QOL, which emphasizes the need for systematic screening for treatment adverse effects and joint pain and implementation of efficient management to improve HRQOL.
文摘We herewith report the rare case of a patient with juvenile rheumatoid arthritis who developed on 2 occasions the clinical picture of an isolated unilateral palsy of the inferior oblique muscle—Brown’s syndrome, following pregnancies, each time in a different eye. Although the eye is frequently involved in rheumatoid arthritis (RA), the Brown’s syndrome is seldom reported in literature with regards to RA.
文摘Background:Treatment of systemic-onset juvenile idiopathic arthritis(So-JIA)is challenging,and the effi cacy of injectable recombinant human tumor necrosis factor type 1 receptor-antibody fusion protein(etanercept)on So-JIA has been controversial.Methods:We retrospectively studied 12 patients with refractory systemic juvenile arthritis treated with etanercept at our hospital in the past 5 years.The 12 patients were divided into a corticosteroid-dependent group(n=7)and an ineffective group(n=5)on the basis of their responses to treatment before the administration of etanercept.Etanercept was added to the treatment without substantially changing the original regimens in general,and doses,and signs of efficacy including alleviation or resolution of symptoms such as high fever,infl ammatory arthropathy,eruption rash,hydrohymenitis,as well as changes in the levels of laboratory infl ammatory markers such as the white blood cell count,erythrocyte sedimentation rate,levels of C-reactive protein and serum ferritin were recorded.Results:Etanercept was withdrawn after the first dose from one patient in the corticosteroid-dependent group because of a systemic allergic rash,and was also withdrawn from one patient in the ineffective group after 2 months of treatment owing to ineffi cacy;the remaining 10 patients completed the entire treatment protocol,at which point etanercept was discontinued.At that time,clinical symptoms and laboratory infl ammatory markers of the remaining patients were within the normal range and the mean dose of prednisone was 0.18 mg/kg per day,an 81%decrease from the mean dose at baseline.At present,the corticosteroid has been discontinued and only methotrexate maintenance treatment is used in 3 patients;the other 7 patients are treated with prednisone and methotrexate maintenance therapy.All of the 10 patients are in a medicated remission with no recurrence.Conclusions:In the treatment of patients with refractory So-JIA,the principles of individual therapy and combinations of drugs should be followed.Etanercept is an important and valid candidate for use in such combined treatment strategies.
