Tumors Constitute a Majority of Total Knee Arthroplasty in Patients <21 Years Old: A United States Nationwide Analysis.

BACKGROUND: Total knee arthroplasty (TKA) is rarely performed in patients under 21 years old, but the frequency of utilization of TKA in this population in the United States is not known. The purpose of this study was to evaluate trends in the use of TKA in patients <21 in the United States. Additionally, we aimed to determine the characteristics of these patients and the hospitals in which this procedure is performed. METHODS: We retrospectively reviewed the Kids' Inpatient Database, a national weighted sample of all inpatient hospital admissions in the United States in patients <21 years of age. We used International Classification of Diseases, Ninth Revision (ICD-9) and ICD-10 codes to identify patients undergoing TKA from 2000 to 2019 and determine a primary diagnosis. Descriptive statistics were calculated using the appropriate sample weights. RESULTS: The total weighted number of TKAs performed in patients <21 years from 2000 to 2019 was 1,535. There were 70.9% of TKAs performed for a primary diagnosis of tumor, and the use of TKA for malignant tumors has increased. In contrast, the use of TKA for inflammatory arthritis/juvenile idiopathic arthritis decreased significantly over the study period. The majority of TKAs were performed in urban teaching hospitals with a large bed size. CONCLUSION: Approximately 1,535 TKAs have been performed in patients <21 years from 2000 to 2019 in the United States. The majority of these procedures were performed for reconstruction after resection of a malignant tumor. The rate of TKA for inflammatory arthritis/juvenile idiopathic arthritis has decreased over the past two decades.

Moving from nature to nurture: a systematic review and meta-analysis of environmental factors associated with juvenile idiopathic arthritis.

OBJECTIVES: JIA is the most common paediatric rheumatic disease, thought to be influenced by both genetics and the environment. Identifying environmental factors associated with disease risk will improve knowledge of disease mechanism and ultimately benefit patients. This review aimed to collate and synthesize the current evidence of environmental factors associated with JIA. METHODS: Four databases (MEDLINE, Embase, Web of Science and Cumulative Index to Nursing and Allied Health Literature) were searched from inception to January 2020. Study quality was rated using the Newcastle-Ottawa Scale. Pooled estimates for each environmental factor were generated using a random-effects, inverse-variance method, where possible. The remaining environmental factors were synthesized in narrative form. RESULTS: This review includes 66 environmental factors from 39 studies (11 cohort and 28 case-control studies) over 45 years. Study sample sizes ranged from 41 to 1.9 million participants. Eight environmental factors from ten studies were meta-analysed. Caesarean section delivery was associated with increased JIA risk [pooled odds ratio (OR) 1.11, 95% CI: 1.01, 1.22]. Conversely, presence (vs absence) of siblings (pooled OR 0.60, 95% CI: 0.44, 0.81) and maternal prenatal smoking (pooled OR 0.70, 95% CI: 0.58, 0.84) were associated with decreased JIA risk. CONCLUSION: This review identifies several environmental factors associated with JIA and demonstrates the huge breadth of environmental research undertaken over five decades. We also highlight the challenges of combining data collected over this period due to limited between study comparability, evolution in healthcare and social practices, and changing environment, which warrant consideration when planning future studies.

Uveítis asociada a la artritis idiopática juvenil / Uveitis associated to juvenile idiopathic arthritis

Objetivo: Caracterizar las uveítis asociadas a la artritis idiopática juvenil. Métodos: Se realizó un estudio observacional, descriptivo, de corte transversal, donde se evaluaron las variables edad, raza, sexo, lateralidad de la uveítis, clasificación anatómica, agudeza visual mejor corregida, presencia de complicaciones y tratamiento. Resultados: Predominaron los mayores de seis años, el sexo femenino y la raza blanca. En cuanto a la lateralidad hubo mayor predominio de las bilaterales, con localización anatómica anterior. En los resultados visuales sobresalieron los que presentaban una agudeza visual mejor corregida ≥ 0,5. Las complicaciones más frecuentes fueron: la pérdida visual, la hipertensión ocular y la queratopatía en banda. Con respecto al tratamiento, la mayoría de los pacientes tenían asociado metotrexate al tratamiento tópico y oral con esteroides. Conclusión: La uveítis asociada a la artritis idiopática juvenil sigue siendo un problema importante de salud en la infancia a pesar de los avances en los programas de atención a esta enfermedad; por tanto, el diagnóstico precoz, el seguimiento estricto y el tratamiento adecuado son los pilares para una mejor evolución(AU)

Objective: Characterize uveitis associated to juvenile idiopathic arthritis. Methods: A cross-sectional observational descriptive study was conducted based on evaluation of the following variables: age, race, sex, laterality of uveitis, anatomical classification, best corrected visual acuity, presence of complications and treatment. Results: A predominance was observed of patients aged over six years, female sex and white race. Bilateral uveitis prevailed, with anterior anatomical location. Patients with a best corrected visual acuity ≥ 0.5 stood out for their visual results. The most common complications were visual loss, ocular hypertension and band keratopathy. Most patients had methotrexate associated to topical and oral treatment with steroids. Conclusion: Uveitis associated to juvenile idiopathic arthritis continues to be an important health problem in childhood, despite the progress in the care of this condition. Therefore, early diagnosis, strict follow-up and appropriate treatment are the pillars of a better evolution(AU)

Long-term outcomes in patients with polyarticular juvenile idiopathic arthritis receiving adalimumab with or without methotrexate.

OBJECTIVES: Long-term safety and efficacy of adalimumab among patients with juvenile idiopathic arthritis (JIA) was evaluated through 6 years of treatment. METHODS: Children aged 4-17 years with polyarticular JIA were enrolled in a phase III, randomised-withdrawal, double-blind, placebo-controlled trial consisting of a 16-week open-label lead-in period, 32-week randomised double-blind period and 360-week long-term extension. Patients were stratified by baseline methotrexate use. Adverse events (AEs) were monitored, and efficacy assessments included JIA American College of Rheumatology (JIA ACR) 30%, 50%, 70% or 90% responses and the proportions of patients achieving 27-joint Juvenile Arthritis Disease Activity Score (JADAS27) low disease activity (LDA, ≤3.8) and inactive disease (ID, ≤1). RESULTS: Of 171 patients enrolled, 62 (36%) completed the long-term extension. Twelve serious infections in 11 patients were reported through 592.8 patient-years of exposure. No cases of congestive heart failure-related AEs, demyelinating disease, lupus-like syndrome, malignancies, tuberculosis or deaths were reported. JIA ACR 30/50/70/90 responses and JADAS27 LDA were achieved in 66% to 96% of patients at week 104, and 63 (37%) patients achieved clinical remission (JADAS27 ID sustained for ≥6 continuous months) during the study. Attainment of JIA ACR 50 or higher and JADAS27 LDA or ID in the initial weeks were the best predictors of clinical remission. Mean JADAS27 decreased from baseline, 22.5 (n=170), to 2.5 (n=30) at week 312 (observed analysis). CONCLUSIONS: Through 6 years of exposure, adalimumab was well tolerated with significant clinical response (up to clinical remission) and a relatively low retention rate.

Juvenile "idiopathic" arthritis (JIA) as a manifestation of food allergy. Case Study / Alergia alimentaria artritis reumatoidea juvenil. Reporte de un caso

ABSTRACT Studies linking type of diet and juvenile idiopathic arthritis (JIA) have variable results and are inconsistent. This case shows an evolution which fulfilled the criteria of JIA, but was diagnosed as food allergy. Case: A seven-year old boy had fever, arthralgia, general malaise, headaches, abdominal pain and rashes. These symptoms were diagnosed as fever of unknown origin (FUO) and probable JIA. There was a stabbing pain in the right iliac fossa. An upper and lower endoscopy were performed and nodular ileocolitis was detected. A hypoallergenic diet was prescribed, in addition to mesalazine and oral corticosteroids. The patient was asymptomatic for 2.5 months and then relapsed with all symptoms after consuming dairy. This JIA case shows the diagnostic phases of food allergy: improvement and recurrence of symptoms with the reintroduction of the allergen (oral challenge=gold standard of food allergy). There is evidence that supports the existence of a gut-joint axis, where the luminal content triggers a series of immunologically mediated reactions that can cause systemic diseases such as JIA and other connective tissue diseases. This case report adds reasonable evidence in support of food allergy as a cause of JIA.

RESUMEN Los estudios que relacionan el tipo de dieta y la artritis idiopática juvenil (AIJ) tienen resultados variables y son inconsistentes. Este caso muestra una evolución que cumplió con los criterios de AIJ, pero fue diagnosticada como alergia alimentaria. Caso: Un niño de siete años tenía fiebre, artralgia, malestar general, dolores de cabeza, dolor abdominal y erupciones cutáneas. Estos síntomas fueron diagnosticados como fiebre de origen desconocido (FUO) y probable AIJ. Hubo un dolor punzante en la fosa ilíaca derecha. Se realizó una endoscopia superior e inferior y se detectó ileocolitis nodular. Se prescribió una dieta hipoalergénica, además de mesalazina y corticosteroides orales. El paciente estuvo asintomático durante 2,5 meses y luego recayó con todos los síntomas después de consumir lácteos. Este caso de AIJ muestra las fases diagnósticas de la alergia alimentaria: mejora y recurrencia de los síntomas con la reintroducción del alergeno (desafío oral = estándar de oro de alergia alimentaria). Existe evidencia que respalda la existencia de un eje de la articulación intestinal, donde el contenido luminal desencadena una serie de reacciones inmunológicamente mediadas que pueden causar enfermedades sistémicas como la AIJ y otras enfermedades del tejido conectivo. Este informe del caso agrega evidencia razonable en apoyo de la alergia a los alimentos como causa de AIJ.

Autoimmune diseases in Turner syndrome: an overview.

Turner syndrome (TS) results from a sex-chromosomal anomaly characterized by presence of one normal X chromosome and the loss of the second X-chromosome in phenotypic females. Autoimmunity has been recognized as one of the more prominent characteristics of TS. The risk of autoimmune diseases in patients with TS is approximately twice as high as in the general female population. The spectrum includes, Hashimoto's thyroiditis, coeliac disease (CD), type 1 diabetes (T1DM), alopecia areata, inflammatory bowel disease, juvenile rheumatoid arthritis and some cutaneous disorders as vitiligo and Halo nevus. This review will address the autoimmune disorders associated with TS, their pathophysiologic mechanisms and clinical characteristics.

Interleukin-6 in juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is a chronic childhood arthritis. Its pathogenesis is very complicated, with the involvement of not only immune cells but various types of parenchymal cells, and is affected by both genetic and environmental predispositions. The clinical spectrum from inflammation to related conditions is largely mediated by cytokines including interleukin (IL)-6. Fluctuations in IL-6 and its related molecules can modulate the pathogenesis and the clinical presentation positively or negatively. The recent clinical impact of IL-6 blockade on JIA has begun a therapeutic paradigm shift. This review describes the characteristics of JIA, mainly focused on IL-6 with the current therapeutic perspective.

Bilateral absence of the cruciate ligaments with meniscal dysplasia: Unexpected diagnosis in a child with juvenile idiopathic arthritis.

Bilateral agenesis of the cruciate ligaments is a rare congenital anomaly. We report a unique case of a young girl who had congenital short femur and diagnosed with polyarticular juvenile idiopathic arthritis (JIA) and later discovered to have congenital absence of both anterior and posterior cruciate ligaments and meniscal dysplasia in both the knees when MRI was performed at 11 years of age. The MRI was performed to evaluate knee laxity and persistent symptoms despite medical management and multiple steroid injections for arthritis treatment. This patient is one of the youngest with congenital absence of both the cruciate ligaments to be treated with ACL reconstruction. We highlight the unique radiographic imaging manifestations of congenital cruciate ligament agenesis and emphasize the role of MRI to confirm and depict additional intraarticular abnormalities.

Juvenile arthritis: current concepts in terminology, etiopathogenesis, diagnosis, and management.

The latest change in terminology from juvenile rheumatoid arthritis (JRA) to juvenile idiopathic arthritis (JIA), established by the International League of Associations for Rheumatology (ILAR), has resulted in some confusion for OMFS and other treating clinicians. JIA comprises a group of systemic inflammatory diseases that result in the destruction of hard and soft tissues in a single or multiple joints. In a significant number of patients, one or both temporomandibular joints (TMJ) are also involved. TMJ disease may be accompanied by pain, swelling, and limitation of motion, as well as mandibular retrognathism, open bite, and asymmetry. The purpose of this article is to provide a review, for the oral and maxillofacial surgeon, of the terminology, etiopathogenesis, diagnosis, and management of children with JIA.

Clinical study of 20 patients with incontinentia pigmenti.

BACKGROUND: Incontinentia pigmenti (IP) is a rare genodermatosis with early prenatal lethality in affected males. Clinical manifestations are usually more exuberant in sporadic than in familial cases. Cutaneous manifestations occur in all sporadic cases and about 96% of familial cases. As well as the skin, other tissues arising from the neuroectoderm may be affected. OBJECTIVES: This study was designed to evaluate dermatologic, dental, neurologic, and ophthalmologic manifestations in patients with IP. METHODS: Findings in IP patients and family members also diagnosed with IP in Porto Alegre, Brazil, during 2003-2012, were analyzed. RESULTS: Thirteen children and seven relatives were diagnosed with IP; 38.4% of cases were familial, and 61.5% were sporadic. Mean ± standard deviation follow-up was 46.08 ± 39.47 months. Frequencies of 100% and 85.7% for dermatologic manifestations, 23.0% and 0% for neurologic manifestations, 62.5% and 71.4% for dental manifestations, and 11.1% and 42.8% for ophthalmologic manifestations were found in affected children and relatives, respectively. Associated diseases include Wilms' tumor, myasthenia gravis, Still's syndrome, and congenital hypothyroidism. CONCLUSIONS: These findings reinforce the heterogeneity of dermatologic findings and the numerous extracutaneous manifestations requiring a multidisciplinary approach. The follow-up of patients with IP is important in the detection of serious associated diseases. The relationships between these disorders and IP raise the need for additional longitudinal studies with longterm monitoring of these patients. The management of IP in clinical practice may benefit from early efforts to detect associated diseases.

Environmental Risk Factors and Early-Life Exposures in Juvenile Idiopathic Arthritis: A Case-Control Study.

OBJECTIVE: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders characterized by chronic arthritis in children with unknown etiology. Although research evaluating environmental or early-life exposures in JIA is scarce, there are data to suggest that infections, smoking exposure, and lack of breastfeeding play a role. This case-control study investigated the association of selected environmental and early-life risk factors with the development of JIA. METHODS: JIA cases were identified at a major pediatric rheumatology outpatient clinic. Each case was asked to identify up to 3 healthy playmates of similar age and same sex to serve as controls. Parents/caregivers of cases and controls completed a questionnaire on selected environmental and early-life exposures. Conditional logistic regression adjusted for age and socioeconomic status was used to determine the odds ratio (OR) for developing JIA with 95% confidence intervals (95% CIs) for the playmate-matched design. RESULTS: Included in the study were 225 JIA cases and 138 controls. Compared to playmate-matched controls, preterm delivery (OR 1.8 [95% CI 1.2-2.7]) was associated with JIA. There was no association between JIA and household smoking or maternal prenatal smoking, breastfeeding, hospitalization with infection in the first year of life, daycare attendance before 6 years of age, household pets, or residential area prior to the onset of JIA. CONCLUSION: There was no association between the previously reported risk factors of smoking, early-life infection, or breastfeeding and development of JIA in this study. The association of preterm delivery with JIA needs to be further studied.

Arthritis as presenting manifestation of acute lymphoblastic leukaemia in children.

BACKGROUND: At disease onset, children with acute lymphoblastic leukaemia (ALL) may present with arthralgia or even signs of arthritis. This might cause misdiagnosis and thereby lead to prolonged diagnostic delay. The present study aimed to identify children with ALL with joint involvement and to compare their characteristics and outcome with children with ALL without joint involvement. METHODS: Case records of 286 children diagnosed with ALL between 1992 and 2013 were reviewed and analysed in this retrospective, descriptive study. RESULTS: Fifty-three (18.5%) children with ALL presented with localised joint pain, and half of them had objective signs of arthritis. The mean number of joints involved was 2.5, most frequently presenting as asymmetric oligoarthritis. The suspected misdiagnosis were reactive arthritis (19/53), osteomyelitis (9/53) and juvenile idiopathic arthritis (8/53). Children with joint involvement had less objective signs of haematological disease. Cytopenia was absent in 24% in children with joint involvement (vs 8% without, p=0.001), 50% had only one cell line affected (vs 21%, p=0.0005) and 44% had no organomegaly (vs 29%, p=0.05). Median diagnostic delay was 4 vs 2 weeks. The 5-year event-free and overall survival was superior for children with joint involvement: 94% vs 87% (p=0.049), and 96% vs 83% (p=0.044). CONCLUSIONS: ALL with joint involvement is a frequent finding (18.5%). The clinical signs of leukaemia are less prominent, but non-articular pain should alert the clinician of a possible diagnosis of leukaemia. The overall and event-free survivals were superior compared with the children without joint involvement.

Autoimmune/auto-inflammatory syndrome induced by adjuvants (ASIA) after quadrivalent human papillomavirus vaccination in Colombians: a call for personalised medicine.

This was a case study in which 3 patients with autoimmune/auto-inflammatory syndrome induced by adjuvants (ASIA) after quadrivalent human papillomavirus vaccination (HPV) were evaluated and described. All the patients were women. Diagnosis consisted of HLA-B27 enthesitis related arthritis, rheumatoid arthritis and systemic lupus erythematous, respectively. Our results highlight the risk of developing ASIA after HPV vaccination and may serve to increase the awareness of such a complication. Factors that are predictive of developing autoimmune diseases should be examined at the population level in order to establish preventive measures in at-risk individuals for whom healthcare should be personalized and participatory.

[Enthesitis in connection with spondyloarthritides]. / Enthesitis im Rahmen von Spondyloarthritiden.

Enthesitis is a frequent manifestation in spondyloarthritis (SpA) and psoriatic arthritis (PsA) and can be found in up to 40% of patients with SpA. Because of the pathognomonic relevance the classification criteria for SpA and PsA use enthesitis as an entrance or secondary criterion. Enthesitis is most frequently localized at the heel but it can occur at any insertion of an enthesis into the bone. When diagnosing enthesitis differential diagnoses should be considered, mechanical-degenerative causes and fibromyalgia in particular should be excluded. The imaging techniques power Doppler ultrasound (PDUS) and magnetic resonance imaging (MRI) are most helpful in making the diagnosis. The therapeutic options for enthesitis are limited. Nonsteroidal antirheumatic drugs (NSARD) and local injections of corticosteroids are recommended. In small clinical trials no efficacy of disease modifying antirheumatic drugs (DMARD) could be demonstrated. In contrast, tumor necrosis factor alpha (TNF-alpha) blockers were shown to be highly effective in randomized controlled trials for SpA and PsA but they are not currently approved for enthesitis only.

Juvenile idiopathic arthritis in relation to maternal prenatal smoking.

OBJECTIVE: Cigarette smoking increases the risk of seropositive adult rheumatoid arthritis. The relationship of smoking with juvenile idiopathic arthritis (JIA), a heterogeneous group of 7 mutually exclusive categories of chronic childhood inflammatory arthritides, is unknown. Our objective was to evaluate the association between JIA and its categories with maternal prenatal smoking. METHODS: This case-control study used International Classification of Diseases, Ninth Revision codes from hospital records to identify 1,196 JIA cases born in Washington state and diagnosed at a quaternary pediatric center from 1997-2010. Controls (n = 5,618) were randomly selected from birth records of children without JIA, frequency matched on birth year. Prenatal smoking exposure was assessed from subjects' birth certificates. Chart review categorized JIA into International League of Associations for Rheumatology categories. Adjusted odds ratios (OR) and 95% confidence intervals (95% CIs) were calculated using logistic regression. RESULTS: We did not observe an increased risk of JIA in relation to maternal prenatal smoking. Prenatal smoking was reported less often among mothers of JIA cases (11%), than among control mothers (17%; OR 0.71 [95% CI 0.58-0.87]), a relationship somewhat more marked for oligoarticular/extended oligoarticular JIA. Although this relationship persisted after adjustment, we cannot rule out that the effect may have been due to residual confounding by socioeconomic status. CONCLUSION: We did not observe an increased risk of JIA or its individual categories with maternal prenatal smoking.