Juvenile idiopathic arthritis

2013 ◽  
Author(s):  
Eileen Baildam
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Multidisciplinary Care ◽  
Chronic Arthritis ◽  
Inflammatory Disorder ◽  
Joint Movement ◽  
Delay In Diagnosis ◽  
Systemic Jia ◽  
Clinical Presentations ◽  
Life Threatening

Juvenile idiopathic arthritis (JIA) is defined as arthritis lasting for 6 weeks or more presenting in childhood at any age up to 17 years. Arthritis is diagnosed clinically by the presence of joint pain, stiffness, and swelling with inflammation limiting the range of individual joint movement. There are subtypes that tend to follow distinct courses and with phenotypes that vary widely from a serious systemic inflammatory disorder of systemic JIA to single-joint monoarthritis. The differential diagnosis of JIA is wide and the best chance of long-term remission is where treatment is started as early as possible. However, there is often delay in diagnosis in childhood and there is no single reliable diagnostic test so pattern recognition is fundamental. There are associated disorders such as silent uveitis that must be screened for and managed as part of essential multidisciplinary care. Systemic JIA is complicated by potentially life-threatening macrophage activation syndrome that is often underdiagnosed but where the diagnosis is based on easy clinical tests and where awareness is vital. This chapter covers descriptions of the classification criteria for chronic arthritis in children, clinical presentations and likely course for the various subtypes.

Juvenile idiopathic arthritis

2013 ◽  
pp. 913-920
Author(s):  
Eileen Baildam
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Multidisciplinary Care ◽  
Chronic Arthritis ◽  
Inflammatory Disorder ◽  
Joint Movement ◽  
Delay In Diagnosis ◽  
Systemic Jia ◽  
Clinical Presentations ◽  
Life Threatening

Juvenile idiopathic arthritis (JIA) is defined as arthritis lasting for 6 weeks or more presenting in childhood at any age up to 17 years. Arthritis is diagnosed clinically by the presence of joint pain, stiffness, and swelling with inflammation limiting the range of individual joint movement. There are subtypes that tend to follow distinct courses and with phenotypes that vary widely from a serious systemic inflammatory disorder of systemic JIA to single-joint monoarthritis. The differential diagnosis of JIA is wide and the best chance of long-term remission is where treatment is started as early as possible. However, there is often delay in diagnosis in childhood and there is no single reliable diagnostic test so pattern recognition is fundamental. There are associated disorders such as silent uveitis that must be screened for and managed as part of essential multidisciplinary care. Systemic JIA is complicated by potentially life-threatening macrophage activation syndrome that is often underdiagnosed but where the diagnosis is based on easy clinical tests and where awareness is vital. This chapter covers descriptions of the classification criteria for chronic arthritis in children, clinical presentations and likely course for the various subtypes.

scholarly journals Long-term follow-up on effectiveness and safety of etanercept in juvenile idiopathic arthritis: the Dutch national register

2008 ◽  
Vol 68 (5) ◽  
pp. 635-641 ◽  
Author(s):  
F H M Prince ◽  
M Twilt ◽  
R ten Cate ◽  
M A J van Rossum ◽  
W Armbrust ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Adverse Events ◽  
Rheumatoid Factor ◽  
Serious Adverse Events ◽  
American College Of Rheumatology ◽  
Systemic Jia ◽  
Long Term Follow Up ◽  
Remission Criteria

Objective:We undertook an observational study to obtain a complete overview of the long-term effectiveness and safety of etanercept in patients with different juvenile idiopathic arthritis (JIA) subtypes.Methods:At baseline we collected patient and disease characteristics of all Dutch patients with JIA who started treatment with etanercept. Disease activity was evaluated (at start of the study, after 3 months and then yearly) according to the JIA core set of the American College of Rheumatology paediatric definition for 30, 50 and 70% improvement (ACR Pedi 30, 50 and 70). Use of etanercept and concomitant drugs was monitored. Adverse events were recorded.Results:We included 146 patients with JIA with a median follow-up of 2.5 years per patient (range 0.3–7.3). JIA subtypes represented: 27% systemic, 8% polyarticular rheumatoid factor positive, 38% polyarticular rheumatoid factor negative, 19% oligoarticular extended, 3% enthesitis-related and 5% psoriatica. Most patients (77%) met the criteria of the ACR Pedi 30 in the first 3 months of treatment. For the majority of patients this improvement was sustained; 53 (36%) of all patients met the remission criteria. No other second-line agents were needed in 43 patients. Although patients with systemic JIA responded initially less to etanercept therapy than patients from other subtypes, those who did respond showed equal effectiveness in the long term. Serious adverse events rate was low (0.029 per patient year).Conclusions:Etanercept is effective and safe in JIA, even for a large proportion of the patients with systemic JIA. The greatest improvement occurred in the first 3 months of treatment, and was sustained for a long time in most patients (up to 75 months).

scholarly journals Multisystem Inflammatory Syndrome in Children and Adolescents Associated with COVID-19: Review

2020 ◽  
Vol 07 (04) ◽  
pp. 13-15 ◽  
Author(s):  
BS Mahapatra ◽  
Keyword(s):  
Early Recognition ◽  
Artery Aneurysm ◽  
Multidisciplinary Care ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Immune Mediated ◽  
Life Threatening ◽  
Adolescent And Young Adults ◽  
Inflammatory Syndrome

Multisystem Inflammatory Syndrome in Children (MIS-C) is a newly recognized multiorgan disease seen in children, adolescent and young adults presumed to be a delayed immune mediated complication of Corona virus 2 (SARS-CoV-2) infection leading to severe acute respiratory syndrome. MIS-C can be associated with life threatening organ dysfunction requiring complex multidisciplinary care. Early recognition is important in order to prevent complication and serious sequalae. Because it is a post infective complication, in most of the cases RT-PCR comes negative though antibodies to COVID-19 are positive. Although SARS-CoV-2 in children are generally mild and nonfatal, there is increasing evidence of MIS-C. Clinical and laboratory features of MIS-C are similar to those of Kawasaki disease like syndrome and Toxic Shock Syndrome. Pathophysiology of MIS-C is still unclear and mainly due to formation of autoantibody and immune complex which activates inflammation. Most of the MIS-C associated with COVID-19, need treatment with ionotropic agents and anticoagulants. The long-term outcome of MIS-C like coronary artery aneurysm formation remain unknown and needs close follow up.

scholarly journals Juvenile Idiopathic Arthritis: Mast Common Rheumatic Disorder in Children - An Overview

1970 ◽  
Vol 27 (2) ◽  
pp. 91-98
Author(s):  
Shahana A Rahman
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Normal Growth ◽  
Chronic Arthritis ◽  
Team Approach ◽  
Effective Management ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
International League ◽  
Made In

Juvenile Idiopathic Arthritis (JIA) is the most common form of chronic arthritis in children and an important cause of both the short term and long term morbidities. JIA is the new terminology proposed by the International League of Associations of Rheumatologists (ILAR). Three separate systems are used currently to classify chronic arthritis in children. These are American College of Rheumatology (ACR) classification, The European League against Rheumatism (EULAR) classification and ILAR classification. The diagnosis of JIA remains a clinical one, and is essentially one of exclusion in addition to suspicion and recognition of patterns. There is no single test for the diagnosis of JIA. The treatment of JIA is rapidly changing. Aims of good management of JIA include: controlling pain and inflammation, preserving function and promoting normal growth and development. Remarkable advances have been made in the management of JIA with the advent of new modalities of treatment. Effective management of JIA needs a multidisciplinary team approach. Even after effective management, about one-third of JIA patients continue to manifest their disease activity into adulthood with serious morbidity and disabilities. DOI: 10.3329/jbcps.v27i2.4252 J Bangladesh Coll Phys Surg 2009; 27: 91-98

scholarly journals Long-term outcomes after penile prosthesis placement for the Management of Erectile Dysfunction: a single-Centre experience

2021 ◽  
Vol 31 (1) ◽  
Author(s):  
Valentine Frydman ◽  
Ugo Pinar ◽  
Maher Abdessater ◽  
William Akakpo ◽  
Pietro Grande ◽  
...  
Keyword(s):  
Patient Satisfaction ◽  
Erectile Dysfunction ◽  
Penile Prosthesis ◽  
Perioperative Outcomes ◽  
Single Centre ◽  
Satisfactory Outcome ◽  
Single Centre Experience ◽  
Life Threatening ◽  
Few Data

Abstract Background Penile prothesis (PP) is the gold-standard treatment of drug-refractory erectile dysfunction (ED). While postoperative outcomes have been widely described in the literature, there are few data about patient satisfaction and intraoperative events. We aimed to assess long-term patient satisfaction and perioperative outcomes after PP implantation in a single-centre cohort of unselected patients using validated scales. Results A total of 130 patients received a PP (median age: 62.5 years [IQR: 58–69]; median International Index of Erectile Function (IEEF-5) score: 6 [IQR: 5–7]). Median follow-up was 6.3 years [IQR: 4–9.4]. Thirty-two (24.6%) patients underwent surgical revision, of which 20 were PP removals (15.4%). Global PP survival rate was 84.6% and previous PP placement was a risk factor for PP removal (p = 0.02). There were six (4.6%) non-life-threatening intraoperative events including two which resulted in non-placement of a PP (1.5%). EAUiaic grade was 0 for 124 procedures (95.4%), 1 for four procedures (3.1%) and 2 for two procedures (1.5%). Of patients who still had their PP at the end of the study, 91 (80.5%) expressed satisfaction. Conclusions PP implantation is a last-resort treatment for ED with a satisfactory outcome. PPs are well accepted by patients.

scholarly journals A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab

2021 ◽  
Vol 10 (6) ◽  
pp. 1329
Author(s):  
Johanna Backhus ◽  
Christian Neumann ◽  
Lukas Perkhofer ◽  
Lucas A Schulte ◽  
Benjamin Mayer ◽  
...  
Keyword(s):  
Disease Activity ◽  
Clinical Activity ◽  
Inflammatory Disorder ◽  
International Consensus ◽  
Steroid Pulse ◽  
Related Disease ◽  
Igg4 Related Disease

Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity. Methods: We retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019. Results: Patients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX. Conclusion: RTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6–9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy.

scholarly journals Enteric Fever Diagnosis: Current Challenges and Future Directions

Pathogens ◽  
2021 ◽  
Vol 10 (4) ◽  
pp. 410
Author(s):  
Durga P. Neupane ◽  
Hari P. Dulal ◽  
Jeongmin Song
Keyword(s):  
Diagnostic Tests ◽  
Enteric Fever ◽  
Asymptomatic Carrier ◽  
Diagnostic Methods ◽  
Effective Control ◽  
Future Directions ◽  
Clinical Presentations ◽  
Global Spread ◽  
Life Threatening ◽  
Pros And Cons

Enteric fever is a life-threatening systemic febrile disease caused by Salmonella enterica serovars Typhi and Paratyphi (S. Typhi and S. Paratyphi). Unfortunately, the burden of the disease remains high primarily due to the global spread of various drug-resistant Salmonella strains despite continuous advancement in the field. An accurate diagnosis is critical for effective control of the disease. However, enteric fever diagnosis based on clinical presentations is challenging due to overlapping symptoms with other febrile illnesses that are also prevalent in endemic areas. Current laboratory tests display suboptimal sensitivity and specificity, and no diagnostic methods are available for identifying asymptomatic carriers. Several research programs have employed systemic approaches to identify more specific biomarkers for early detection and asymptomatic carrier detection. This review discusses the pros and cons of currently available diagnostic tests for enteric fever, the advancement of research toward improved diagnostic tests, and the challenges of discovering new ideal biomarkers and tests.

scholarly journals Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jeong-Seon Lee ◽  
Joong-Gon Kim ◽  
Soyoung Lee
Keyword(s):  
Polyarteritis Nodosa ◽  
Clinical Manifestations ◽  
Long Term Outcomes ◽  
Childhood Onset ◽  
Laboratory Findings ◽  
Clinical Presentations ◽  
Long Term Prognosis ◽  
Digital Amputation

AbstractChildhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.

scholarly journals Phenylketonuria and juvenile idiopathic arthritis: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Ting Ting Zhu ◽  
Jin Wu ◽  
Li Yuan Wang ◽  
Xiao Mei Sun
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Case Report ◽  
Autoimmune Diseases ◽  
Hip Joint ◽  
Metabolic Disorder ◽  
Rare Case ◽  
Molecular Data ◽  
Methotrexate Therapy ◽  
Case Presentation

Abstract Background Phenylketonuria (PKU) is a genetic metabolic disorder in which patients have no ability to convert phenylalanine to tyrosine. Several autoimmune diseases have been reported to combine with PKU, co-existent of PKU and Juvenile Idiopathic Arthritis (JIA) has not been presented. Case presentation The girl was diagnosed with PKU at the age of 1 month confirmed by molecular data. At the age of 3.5 years, she presented with pain and swelling of her right ankle, right knee, and right hip joint. After a serial of examinations, she was diagnosed with JIA and treated with a nonsteroidal anti-inflammatory drug. Conclusions We report a rare case of a 4-year-old girl with PKU and JIA, which supports a possible interaction between PKU and JIA. Long-term metabolic disturbance may increase the susceptibility to JIA. Further chronic inflammation could alter the metabolism of tryptophan and tyrosine to increase blood Phe concentration. In addition, corticosteroid and methotrexate therapy for JIA may increase blood Phe concentration.

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