History Of Juvenile Idiopathic Arthritis Research Articles

Blau Syndrome With Delayed Cutaneous Manifestations: A Case Report.

Blau syndrome is a rare familial autoinflammatory disorder characterized by the triad of granulomatous dermatitis, polyarthritis, and uveitis. Blau syndrome exhibits an autosomal dominant inheritance pattern and can be caused by a gain-of-function mutation in nucleotide-binding oligomerization domain 2 (NOD2), a member of the NOD-like receptor family of pattern recognition receptors. Mutations in NOD2 cause upregulation of inflammatory cytokines and resultant autoinflammation. Because of the rarity of this condition and early onset of symptoms, Blau syndrome may be misdiagnosed as juvenile idiopathic arthritis. We present a case of a 37-year-old male patient with a long-documented history of juvenile idiopathic arthritis and uveitis, who developed an asymptomatic eruption of pink papules on the trunk and upper extremities. A biopsy demonstrated noncaseating, well-formed dermal granulomas with relatively sparse lymphocytic inflammation and Langerhans-type giant cells. Genetic testing confirmed a mutation in NOD2. Based on the patient's clinical history, histologic findings, genetic testing, the diagnosis of Blau syndrome was made.

Unusual Presentation of Acute Coronary Syndrome in a 22-Year-Old Male with Juvenile Idiopathic Arthritis: A Case Report

This case report documents the clinical journey of a 22-year-old male who was initially diagnosed with Rheumatoid Arthritis (RA) and later, at the age of 11, with Juvenile Idiopathic Arthritis (JIA). The patient manifested atypical symptoms, primarily marked by sudden and intense chest pain accompanied by sweating, leading to a collapse during his emergency room visit. Medical evaluation revealed an extensive myocardial infarction affecting the anterior wall. The patient underwent a successful percutaneous coronary intervention targeting the left anterior descending artery. This case underscores the rare incidence of acute coronary syndrome in young patients with a history of JIA.

Acute Lymphoblastic Leukaemia (ALL) with History of Juvenile Idiopathic Arthritis (JIA)

Backgrounds: Distinguishing leukemia from Juvenile Idiopathic Arthritis (JIA) is sometimes very difficult. Children with leukemia can also present with complaints of the bones and muscles and many children with leukemia initially present without blast cells in the peripheral blood. Case Reports: A boy aged 8 years 7 months with complaints of joint pain since 6 months before he was admitted to the hospital. Pain was felt intermittently, there was no complaint of fever or bleeding. physical examination, blood pressure 100/70mmHg, pulse 100times/minute, respiratory rate 24times/minute, temperature 36.70C, obtained anemia and splenomegaly. Laboratory tests showed WBC 242.00 x 103/μL, absolute lymphocytes 98.60 x 10 3/μL, RBC 1.54 x 106/μL, HGB 6.02g/dL, PLT 78.09 x 103/μL, CRP 21.65mg/dL, ANA(IF )# negative, RF negative, C3 Complement 150.6mg/dL. Bone Marrow Aspiration (BMA) Acute Lymphoblastic Leukemia (ALL) L2. Conclusion: A boy aged 8 years and 7 months with an initial diagnosis of Juvenile Idiopathic Arthritis, after 3 months of treatment the patient was diagnosed with acute lymphoblastic leukemia based on the results of bone marrow aspiration examination. Currently the patient is undergoing a Leukemia therapy protocol.

Fitness to Serve in the Armed Forces and Internal Medicine: A Retrospective Study.

Assessment of the medical fitness to serve in the armed forces has two objectives: to prevent the military operations from being jeopardized by a medical issue, and to protect soldiers from the sequelae of diseases that could become complicated in the operational field, especially in overseas operations where soldiers are exposed to a remote setting and a long evacuation time. Little is known about fitness decisions for soldiers with systemic or autoimmune diseases. Therefore, we conducted a single-center retrospective study of internal medicine fitness decisions. All the fitness decisions discussed from September 2019 to December 2020 in our department of internal medicine were reviewed. Gender, age, army or service, rank, garrison and health topic were collected from the medical files. Our Military Hospital local ethics committee, in accordance with the French law, approved this study. There were 41 cases, involving 31 men and 10 women (mean age: 31 years), presenting with autoimmune or systemic diseases, metabolic disorders, thrombophilia, congenital or acquired malformations or organ failure, miscellaneous nephropathies, or hemogram abnormalities. Four patients were taking immunosuppressive agents, 3 biologics, and 4 anticoagulants. Among the 15 civilians requiring medical fitness assessment to enlistment, 6 were declared fit. They presented with a history of juvenile idiopathic arthritis with intermediate uveitis without relapse for 7 years, Mayer-Rokitansky-Küster-Hauser syndrome type II with ectopic kidney, solitary kidney with normal renal function and with hypertension, isolated proteinuria, proteinuria with microscopic hematuria, and muscular fibrolipoma with a history of surgical treatment of a vascular malformation. Among the 26 patients already enlisted in the armed forces, 9 were referred for assessment of medical fitness to serve overseas. Two soldiers were assessed as fit without restrictions; one presented with a history of a single episode of deep vein thrombosis after surgery, and the other had a history of monoclonal gammopathy of renal significance without relapse and without treatment for 8 years. Four soldiers were assessed as fit only for overseas territories with sanitary structures similar to mainland France. They presented with immunoglobulin A (IgA) nephropathy and treatment with angiotensin-converting enzyme inhibitor, mevalonate kinase deficiency and treatment with anakinra, chronic idiopathic thrombocytopenic purpura, and history of unilateral partial renal infarction. The 17 other soldiers were referred for dispensation, long-sickness leave granting, or for specification toward administrative coding of their disease. We have described the first exhaustive study of specialized fitness decisions referred to an internal medicine department. One-third of the referred patients were declared fit to serve in the armed forces. Further studies are needed to confirm these results, as our study was monocentric. Fitness decisions must take into account the disease, the treatment, and the operational field characteristics. Soldiers with systemic diseases controlled by immunosuppressive agents can serve in tropical areas if they can reach adequate sanitary structures in a short time. The knowledge of systemic diseases as well as the skillfulness of the internists, which are regularly projected to the operational fields, allows them to provide pragmatic fitness expertise to myriad complex situations.

Multisystem Autoimmune Inflammatory Disease, Including Colitis, Due to Inborn Error of Immunity

Our understanding of inflammatory bowel disease is changing as we identify genetic variants associated with immune dysregulation. Inflammatory bowel disease undetermined, even when diagnosed in older children and adolescents, in the setting of multiple inflammatory and infectious diseases should raise the suspicion of complex immune dysregulation with a monogenic basis. We report a case of inflammatory bowel disease undetermined triggered by exposure to a nonsteroidal antiinflammatory drug in a 16-year-old girl with a background history of juvenile idiopathic arthritis, cytopenias, recurrent respiratory tract and middle ear infections, and esophageal candidiasis. Immunologic assessment included measurement of immunoglobulin levels, lymphocyte immunophenotyping, B-cell functional tests, and whole-exome sequencing. Laboratory investigation revealed defects of humoral immunity, including mild persistent hypogammaglobulinemia affecting all 3 isotypes and absent isohemagglutinins. Whole exome sequencing revealed a heterozygous TNFRSF13B (Tumor Necrosis Factor Receptor Superfamily Member 13B, or Transmembrane Activator and Calcium-modulating cyclophilin ligand Interactor, TACI) gene variant, which is associated with common variable immunodeficiency and the development of autoimmune diseases. In conclusion, a clinical history of recurrent infections, atypical histologic features of inflammatory bowel disease, additional autoimmune manifestations, and an inadequate response to conventional therapy should prompt the physician to refer to an immunologist with the query of inborn error of immunity. We report how extensive immune evaluation and genetic diagnosis can individualize care and facilitate a multidisciplinary team approach.

Displacement of Ex-PRESS Shunt Head Outside the Conjunctiva in a Uveitis Patient: A Case Report

Ex-PRESS mini shunt is recently involved in trabeculectomy surgery with a good outcome. We present a 14-year-old male with a history of juvenile idiopathic arthritis, non-granulomatous anterior uveitis, and secondary glaucoma operated with trabeculectomy Ex-PRESS shunt in his right eye. The patient developed extrusion of the implant head outside the conjunctiva after 4 years of surgery. The patient admitted to the hospital with removal of Ex-PRESS shunt under general anesthesia with the suturing of the scleral defect and closure of the conjunctiva.

Health State Utility Values in Juvenile Idiopathic Arthritis: What is the Evidence?

The objectives of this systematic review were to identify health state utility values (HSUV) of children and adults with juvenile idiopathic arthritis in the literature and to assess whether HSUV were appropriately reported and could be used to inform parameter inputs for a model-based cost-utility analysis to inform decision making. MEDLINE, EMBASE, PsycINFO, EconLit and CINAHL databases were searched in July 2019. Inclusion criteria were studies using preference-based instruments, targeting children or adults with juvenile idiopathic arthritis, and in the English language. The quality of studies was assessed using a modified checklist that included relevant sources of bias and assessment of quality of HSUV valuation and measurement. A descriptive analysis was conducted, including assessment on reporting of population characteristics and stratification of HSUV by potential health states or population subgroup. From 620 identified articles, ten reported HSUV. Seven studies reported HSUV of children with juvenile idiopathic arthritis, and three of adults with a history of juvenile idiopathic arthritis. Population disease activity status and drug treatment were reported in less than half of the studies. Six (out of ten) studies stratified HSUV results for at least one of the potential health state categories, but they represent very specific situations or interventions (e.g. patients receiving different types of physiotherapy or treated with etanercept over time). We have identified critical gaps in the literature reporting HSUV in patients with juvenile idiopathic arthritis including a lack of HSUV measures for distinct health states, particularly in adults with a history of juvenile idiopathic arthritis. The current reported HSUV data in juvenile idiopathic arthritis are insufficient for a full cost-utility analysis with a short or lifetime horizon.

Regional differences in temporomandibular joint inflammation in patients with juvenile idiopathic arthritis: a dynamic post-contrast magnetic resonance imaging study

The purpose was to determine whether there are regional differences in temporomandibular joint (TMJ) inflammation in patients with juvenile idiopathic arthritis (JIA). This was a retrospective study of all patients with dynamic, contrast-enhanced magnetic resonance imaging through the TMJs at Massachusetts General Hospital between January 2015 and July 2016. The patient cohort included those with a history of JIA and control patients who underwent MRI for other routine clinical purposes. TMJ inflammation was quantified as the difference between post-gadolinium and pre-gadolinium articular T1 signal intensity normalized to post-gadolinium signal intensity of the longus capitis muscle. TMJ enhancement profiles were generated for the lateral, central, and medial portions of the TMJ. Regional differences in TMJ enhancement were investigated using basic descriptive statistics. Medial edge enhancement of the TMJs was highest in symptomatic JIA joints, followed by asymptomatic JIA, then control joints. Medial edge enhancement was a significant discriminator between symptomatic JIA TMJs and control joints (P = 0.0001), between symptomatic and asymptomatic JIA TMJs (P = 0.0003), and between asymptomatic JIA TMJs and controls (P = 0.0019). A shift in distribution of TMJ enhancement towards the medial edge that was seen uniquely in both asymptomatic and symptomatic JIA TMJs compared to control joints was found. This suggests a pattern of worsening medial edge inflammation with disease.

PRM94 - Quality of Life of Adults with a History of Juvenile Idiopathic Arthritis: A Systematic Review

Juvenile idiopathic arthritis (JIA) is a rheumatologic disease affecting 1 in 1,000 children resulting in life-long disability. New treatments for JIA have the potential to alter disease course. However, a challenge in economic evaluations of JIA drug treatment is to obtain quality of life (QoL) data, by JIA subtype, related to the transition of care from childhood to adulthood to inform life-time horizon cost effectiveness models. Our objective is to conduct a systematic review to identity QoL of adults with JIA. MEDLINE and EMBASE databases were searched. Studies in English that collected primary data evaluating QoL in adults with a history of JIA were included. Abstracts and full-text were screened independently by 2 reviewers. Data extracted included publication year, QoL instrument, QoL results, and sample size. We identified 1031 articles, and included 15 published between 1997-2017. Fourteen unique questionnaires were completed by 4023 individuals. QoL instruments included 3 generic multi-dimensional, 6 generic uni-dimensional (dimensions: pain, fatigue, well-being, vision, coping), 2 disease-specific multi-dimensional, and 2 generic multi-dimensional utility-based instruments. The most commonly used were the Health Assessment Questionnaire (n=10 studies), and SF-36 (n=9). Disease-specific instruments were Childhood Health Assessment Questionnaire (n=2), and Arthritis Impact Measurement Scale (n=1). Two studies reported EQ5D index scores: 0.902 (range:0.03-1; using German value set), and 0.8 (range:0.1-1; value set undefined). There was significant variability in reporting of QoL results, even within instrument. Few studies reporting utility data were identified, and utilities were not stratified by disease status or subtype. Data reported are not sufficient to inform life-time horizon models. Generic QoL instruments were used more often than disease-specific instruments. The heterogeneity in reporting QoL results highlights the need for standardization. It remains a need for rigorous QoL assessment during and after the transition of care to inform economic evaluations of early interventions.

Influence of juvenile idiopathic arthritis on the quality of life of young adults in the transition period to adult rheumatologic care

Background Young adults with juvenile idiopathic arthritis (JIA) often have an active disease with significant functional impairment in adulthood that can affect their physical and mental functions Aim To determine the impact of JIA on quality of life (QoL) in young adults with JIA during the transition healthcare Materials and methods The cross-sectional study of 89 young adults aged 16 to 22 years with a history of JIA regardless of the presence or absence of active inflammation at the time of the survey was performed in the Oleksandrivsky Central Clinical Hospital in Kyiv, Ukraine in the period between April 2015 and February 2017. 25 age- and sex-matched controls (without rheumatic disease) were included. There was performed an evaluation of the age at disease onset, duration, JIA activity, received therapy and quality of life Results Out of 89, 37 (41,6%) patients were considered to be in remission. Juvenile idiopathic arthritis had a large impact on the physical scales of quality of life. Patients with JIA had worse (p <0.001) physical health in comparison with the control group. The pain was the most important factor affecting the quality of life in cases of juvenile idiopathic arthritis. The indices responsible for the physical (p<0.001), role (borderline significance, p=0.04) functioning, and intensity of pain (p<0.001) were decreased, compared with the control group. However, the indicators responsible for psychological function in patients with JIA did not differ from the control group. The correlation analysis revealed significant negative association between disability severity (HAQ) and physical function (r=-0,56, p<0.001), role function (r=-0,33, p<0.001), pain intensity (r=-0,60, p<0.001), general health (r=-0,40, p=0.01), vital activity (r=-0,46, p<0.001), social function (r=-0,48, p<0.001), mental health (r=-0,42, p<0.001) Conclusion In our transitional cohort of patients at the era of biological therapies, juvenile idiopathic arthritis had a larger effect on the physical than mental SF-36 subscale. The pain was the main factor influencing the quality of life

Optimization of Quantitative Dynamic Postgadolinium MRI Technique Using Normalized Ratios for the Evaluation of Temporomandibular Joint Synovitis in Patients with Juvenile Idiopathic Arthritis.

MR imaging has been shown to be useful in the diagnosis of juvenile idiopathic arthritis of the temporomandibular joint. Prior MR imaging approaches have relied mainly on the subjective interpretation of synovial enhancement as a marker for synovial inflammation. Although, more recently, several attempts have been made to quantify synovial enhancement, these methods have not taken into account the dynamic enhancement characteristics of the temporomandibular joint and the effect of sampling time. Our aim was to develop a clinically feasible, reproducible, dynamic, contrast-enhanced MR imaging technique for the quantitative assessment of temporomandibular joint synovitis in patients with juvenile idiopathic arthritis and to study the effect of sampling time on the evaluation of synovitis. This was a retrospective study of all patients who had dynamic, contrast-enhanced coronal T1 3T MR imaging through the temporomandibular joint at our institution between January 1, 2015, and July 8, 2016. Patients in this cohort included those with a history of juvenile idiopathic arthritis and control patients who underwent MR imaging for other routine, clinical purposes. Synovial enhancement was calculated for each temporomandibular joint using 3 different types of equations termed normalization ratios. The enhancement profiles generated by each equation were studied to determine which provided the best discrimination between affected and unaffected joints, was the least susceptible to sampling errors, and was the most clinically feasible. A ratio of synovial enhancement (defined as the difference between the postgadolinium and the pregadolinium T1 signal of the synovium) to the postgadolinium signal of the longus capitis provided the best discrimination between affected and unaffected joints, the least susceptibility to sampling error, and was thought to be the most clinically feasible method of quantification of synovial inflammation. Additional synovial enhancement ratios studied did not provide the same level rates of discrimination between the affected and unaffected joints and were thought to be too temporally variable to provide reliable clinical use. We provide a robust, reproducible, dynamic gadolinium-enhanced MR imaging technique for the quantitative assessment of temporomandibular joint synovitis in patients with juvenile idiopathic arthritis.

Impact of Uveitis on Quality of Life in Adult Patients With Juvenile Idiopathic Arthritis.

To establish the impact of uveitis on the quality of life (QoL) in adult patients with juvenile idiopathic arthritis (JIA). Adult patients with a history of JIA, both with (n = 31) or without (n = 51) chronic anterior uveitis, were included. Their scores on 3 validated QoL questionnaires (National Eye Institute Visual Functioning Questionnaire [NEI VFQ-25], Medical Outcomes Study 36-Item Short Form health survey [SF-36], and EuroQol 5-domain questionnaire [EQ-5D]) were analyzed to find factors that could influence QoL. The median overall composite score (OCS) of the NEI VFQ-25 was significantly worse in the uveitis group compared to the non-uveitis group (respectively, 83.4 [range 15.2-94.7] and 94.9 [range 46.3-100]; P < 0.001). Nearly all subscale scores were lower in patients with uveitis than in patients without uveitis (P < 0.001 for all). After adjusting for duration of arthritis, JIA subtype, arthritis onset before or after 1990, and the use of systemic immunomodulatory medication, the QoL was still worse in patients with uveitis (NEI VFQ-25 OCS regression coefficient = -11.7; P = 0.002). No significant differences were found between the groups for the SF-36 and the EQ-5D. In the total JIA group, the use of systemic medication appeared to negatively influence some general QoL scores. Having a history of uveitis has a substantial negative effect on the vision-related QoL in JIA in adulthood, despite good visual acuity. General QoL scores did not differ between uveitis and non-uveitis patients, but the use of systemic immunomodulatory treatment, independent of uveitis, did negatively influence general QoL scores in adult JIA patients.

Juvenile Idiopathic Arthritis in Adults: Long-Term Observation of Ukrainian Patients

The assessment of long-term outcome of functional disability and disease activeness in adult patients with juvenile idiopathic arthritis appears to be complicated due to the absence of a unified approach to the classification and estimation of disease activeness, as well as the loss of supervision over a patient because of remission or his/her transition from pediatric to adult rheumatic service. The objective of the research was to determine how adults with the history of juvenile idiopathic arthritis fulfill the classification criteria for adult rheumatic diseases, as well as to assess activeness of these diseases, the degree of functional disorders, and social activeness of patients in Ukraine. Materials and methods. Patients with juvenile idiopathic arthritis older than 18 years and with more than 3 years of disease duration living in different parts of Ukraine were included into the study. Data regarding sociodemographic features, fulfillment of adult classification criteria, Health Assessment Questionnaire, articular and extra-articular Juvenile Arthritis Damage Index and disease activity were analyzed.Results. We observed 122 adult patients with the history of juvenile idiopathic arthritis irrespective of the presence of active inflammation at the moment of the examination. This group included patients from different regions of Ukraine diagnosed with juvenile idiopathic arthritis during 1984-2013. An adult rheumatologist examined all patients and the diagnosis was revised according to the adult classification of rheumatic diseases. Typical diagnostic criteria for rheumatoid arthritis were estimated in 32.8% of patients, ankylosing spondylitis – in 31.1% of patients, undifferentiated arthritis – in 13.9% of patients, Still’s disease – in 4.9% of patients, psoriatic arthritis – in 0.8% of patients, steady clinical laboratory remission – in 16.5% of patients. Most patients (81.8%) with rheumatoid factor positive polyarticular juvenile idiopathic arthritis fell under rheumatoid arthritis criteria in adulthood, and in 85% of patients with enthesitis-related arthritis as well as 53.8% of patients with extended oligoarthritis ankylosing spondylitis developed in adulthood. 68.8% of patients with systemic juvenile idiopathic arthritis, 68% of patients with rheumatoid factor negative polyarthritic subtype and 55% of patients with enthesitis-related arthritis had disability and incapacitation. Minimal disorders of the patients’ general condition according to the Health Assessment Questionnaire in adult age were found in most subtypes of juvenile idiopathic arthritis classified according to the International League of Associations for Rheumatology (extended and persistent oligoarthritis, rheumatoid factor positive polyarthritis, systemic subtype); moderate disorders of the general condition were found in enthesitis-related arthritis and rheumatoid factor negative polyarthritis. Side effects of juvenile idiopathic arthritis according to the articular Juvenile Arthritis Damage Index included severe articular damage being most frequently found in systemic and rheumatoid factor positive polyarthritis subtypes of juvenile idiopathic arthritis, while side effects of juvenile idiopathic arthritis according to the extra-articular Juvenile Arthritis Damage Index included extra-articular damage being found in systemic and rheumatoid factor negative polyarthritis subtypes of juvenile idiopathic arthritis, that was confirmed by the assessment of physical health according to the Short Form Health Survey-36, which was the worst in patients with systemic (40.3±12.6) and rheumatoid factor negative polyarthritis (38.9±9.4) subtypes of juvenile idiopathic arthritis.Conclusions. Further research of remote consequences of juvenile idiopathic arthritis in adult age and long-term observation of such patients require a detailed study to improve diagnostics and provide adequate treatment of rheumatic diseases with juvenile onset in adult age.

Frequency and morbidity of temporomandibular joint involvement in adult patients with a history of juvenile idiopathic arthritis

Purpose Most patients with juvenile idiopathic arthritis (JIA) have temporomandibular joint (TMJ) involvement, but little is known about the natural history of TMJ disease as these children enter adulthood. The purpose of this study was to evaluate adults with a history of JIA to document the frequency and severity of TMJ abnormalities and morbidity. The authors hypothesized that most would have persistent TMJ disease as adults. Materials and Methods This cross-sectional study included adults (>19 years of age) with JIA who were managed at Boston Children's Hospital (BCH) as children and at Brigham and Women's Hospital (BWH) as adults. History of a TMJ problem was not considered for enrollment. Patients completed a questionnaire and underwent physical examination and maxillofacial cone-beam computed tomography (CBCT). Additional data were obtained from medical records. Associations between TMJ abnormalities at CBCT and arthritis history, TMJ pain and function, facial asymmetry, malocclusion, and cephalometric analysis were examined. Results Of 129 eligible patients contacted, 21 (42 TMJs) were enrolled. Mean age was 26.0 ± 6.1 years and mean duration of care for JIA at the BCH and BWH was 13.7 ± 6.5 years. TMJ pain was present in 62% of patients (n = 13); 43% (n = 9) had a TMJ functional limitation and 76% (n = 16) had lower facial asymmetry. Abnormalities were found in the TMJs on 55% of CBCT scans, with 79% showing bilateral deformities. There was at least 1 cephalometric measurement of mandibular size or position that was more than 1 standard deviation beyond normal in 81% of patients (n = 17). Only 4 patients (19%) had previously been evaluated for a TMJ problem. Conclusion TMJ abnormalities and related morbidity are common in adult patients with a history of JIA. Therefore, an early screening protocol for TMJ involvement in children with a new diagnosis of JIA would be beneficial and long-term follow-up into adulthood should be routine.

Frequency and Morbidity of Temporomandibular Joint Involvement in Adult Patients With a History of Juvenile Idiopathic Arthritis

Most patients with juvenile idiopathic arthritis (JIA) have temporomandibular joint (TMJ) involvement, but little is known about the natural history of TMJ disease as these children enter adulthood. The purpose of this study was to evaluate adults with a history of JIA to document the frequency and severity of TMJ abnormalities and morbidity. The authors hypothesized that most would have persistent TMJ disease as adults. This cross-sectional study included adults (>19years of age) with JIA who were managed at Boston Children's Hospital (BCH) as children and at Brigham and Women's Hospital (BWH) as adults. History of a TMJ problem was not considered for enrollment. Patients completed a questionnaire and underwent physical examination and maxillofacial cone-beam computed tomography (CBCT). Additional data were obtained from medical records. Associations between TMJ abnormalities at CBCT and arthritis history, TMJ pain and function, facial asymmetry, malocclusion, and cephalometric analysis were examined. Of 129 eligible patients contacted, 21 (42 TMJs) were enrolled. Mean age was 26.0 ± 6.1years and mean duration of care for JIA at the BCH and BWH was 13.7 ± 6.5years. TMJ pain was present in 62% of patients (n= 13); 43% (n= 9) had a TMJ functional limitation and 76% (n= 16) had lower facial asymmetry. Abnormalities were found in the TMJs on 55% of CBCT scans, with 79% showing bilateral deformities. There was at least 1 cephalometric measurement of mandibular size or position that was more than 1 standard deviation beyond normal in 81% of patients (n= 17). Only 4 patients (19%) had previously been evaluated for a TMJ problem. TMJ abnormalities and related morbidity are common in adult patients with a history of JIA. Therefore, an early screening protocol for TMJ involvement in children with a new diagnosis of JIA would be beneficial and long-term follow-up into adulthood should be routine.

Birth Outcomes in Women with a History of Juvenile Idiopathic Arthritis.

To determine whether children born to women who had juvenile idiopathic arthritis (JIA) had more adverse birth outcomes than children born to mothers who never had JIA. Our cohort study used data from physician billing and hospitalizations covering the province of Quebec, Canada. We identified all women with JIA with a first-time birth between January 1, 1983, and December 31, 2010, and assembled a control cohort of first-time mothers without JIA from the same administrative data, matching 4:1 for date of first birth, maternal age, and area of residence. We compared outcomes (stillbirth, prematurity, small for gestational age, and major congenital anomalies) in the JIA versus non-JIA groups using logistic regression. Mean age at delivery was 24.7 years in the JIA group (n = 1681) and 25.0 years for the non-JIA group (n = 6724). Women who had JIA were at higher risk for a premature baby [adjusted relative risk (RR) 1.20, 95% CI 1.01-1.42], a baby small for gestational age (adjusted RR 1.19, 95% CI 1.04-1.37), and a child with a congenital malformation (adjusted RR 6.51, 95% CI 5.05-8.39). Neural tube defects were higher in the JIA offspring: 1.61% (95% CI 1.11-2.33) versus 0.03% (95% CI 0.01-0.11) in the non-JIA group, as were congenital heart defects: 1.07% (95% CI 0.68-1.69) versus 0.58% (95% CI 0.42-0.79). Most women with JIA will deliver a normal baby, even though they are at higher risk for having a child with adverse birth outcomes. Research is needed to understand pathophysiologic mechanisms and to investigate the effects of medications during childhood and youth on future birth outcomes.

ANTI GLUTAMIC ACID DECARBOXYLASE ANTIBODY MEDIATED ENCEPHALOPATHY WHILE ON ETANERCEPT IN A PATIENT WITH MULTIPLE AUTOIMMUNE DISEASES

A 39 year old lady presented with an altered mental state and unsteadiness of gait. She had a history of juvenile idiopathic arthritis, autoimmune thyroiditis and alopecia, protein S deficiency...

Candidiasis of the Small Bowel Causing Gastrointestinal Bleed

Purpose: Recognize small bowel Candidiasis as a rare cause of gastrointestinal bleed. Case Report: A 31-year-old woman with a history of juvenile idiopathic arthritis, not on chronic immunosuppression, was initially admitted to an outside hospital with acute malaise, arthralgias and rash complicated by the development of ARDS, requiring transfer to our hospital for further workup and management. Upon presentation, she was diagnosed with Adult-onset Still's disease and was treated with high doses of IV methylprednisolone. On hospital day #7, she developed hemoccult negative watery diarrhea of unclear etiology. The infectious workup, including cultures, was negative. Despite aggressive ICU management including multiple broad spectrum antibiotics, she remained critically ill with multi-organ dysfunction and was eventually initiated on Anakinra for treatment of her rheumatologic condition. She did experience overall clinical improvement, but her loose stools persisted. On hospital day #20, she developed melena and, subsequently, transfusion-dependent anemia. An esophagogastroduodenoscopy revealed a diffusely edematous and erythematous small bowel with active oozing of blood from the duodenum extending into the mid-jejunum. This mucosal appearance was also noted in the terminal ileum on colonoscopy. Biopsies from throughout the small bowel were positive for fungus, suggestive of Candida. Gastric and colonic biopsies were negative for fungus. IV fluconazole was initiated with subsequent improvement in her melena and anemia. Discussion: Small bowel Candidiasis has been recognized as a rare cause of watery diarrhea in immunocompromised patients. Rare case reports have documented small bowel fungal infections leading to gastrointestinal bleeding. In a 1992 autopsy study of 890 patients with malignant disease, Prescott et al. described 14 cases of small bowel fungal infections (Candida or Aspergillus spp), with only three Candidal infections associated with melena. Only one case of fungal infection was confined solely to the small intestine. Unlike previously described cases of melena caused by small bowel Candidiasis, our case demonstrates that small bowel Candidiasis can occur in the absence of underlying malignancy or extraintestinal Candidal infection. Though rare, small bowel Candidiasis should be considered in severely immunocompromised, acutely ill, hospitalized patients who develop clinically significant gastrointestinal bleeding even in the absence of underlying malignancy. Prompt recognition of this uncommon disease along with initiation of anti-fungal therapy is necessary to limit further bleeding and prevent potential complications such as small bowel perforation.

Bone Health in Adult Men and Women with a History of Juvenile Idiopathic Arthritis

Our aim was to determine areal bone mineral density (BMD(a)) and disease-related factors linked with BMD(a) in adults with a history of juvenile idiopathic arthritis (JIA). Men and women with a history of JIA attending a young adult rheumatology clinic in Newcastle, UK, underwent dual energy x-ray absorptiometry (DEXA) of the lumbar spine and total hip. Information was obtained about disease duration and subtype, previous treatment including corticosteroid and methotrexate therapy, and large-joint replacement. Subjects completed the modified Health Assessment Questionnaire (HAQ). Blood was taken for assessment of C-reactive protein, erythrocyte sedimentation rate, and rheumatoid factor (RF). Seventy-one women and 16 men, mean age 28.7 and 31.4 years, and mean disease duration 20.6 and 24.0 years, respectively, were studied. Mean BMD(a) was 0.982 (Z-score = -0.328; 95% CI -0.657, 0.001) and 1.028 g/cm(2) (Z-score = -0.251; 95% CI -1.266, 0.764) in women and men, respectively, at the spine and 0.817 (Z-score = -0.542; 95% CI -0.975, -0.109) and 0.857 g/cm(2) (Z-score = -0.176; 95% CI -2.323, 1.971) at the hip. After adjusting for age and sex, increasing HAQ score was associated with both lower spine BMD(a) and hip BMD(a). Compared with patients with oligoarticular disease, those with enthesitis-related arthritis had higher BMD(a) at the spine, while those with extended oligoarticular and polyarticular RF-negative disease had lower hip BMD(a). Oral corticosteroids and the presence of a large-joint replacement were associated with lower BMD(a) at both the spine and hip. There was a trend toward low BMD(a) in women with a history of JIA. These patients may be at risk of the complications of osteoporosis including fragility fractures and should be considered for targeted preventive measures.

The Dilemma of Central Serous Retinopathy, a Corticosteroid-induced Complication, in Patients with Ocular Inflammatory Disease

To the Editor: Rheumatologists sometimes collaborate with ophthalmologists in the management of patients with ocular inflammatory disease. Corticosteroids are often a valuable component of this management. Ophthalmic complications associated with corticosteroid usage include cataract and glaucoma. Corticosteroids have also been implicated in another important cause of visual loss, central serous retinopathy (CSR)1,2, a disorder characterized by neurosensory detachment of the retina affecting the macula. The combination of CSR, best managed by reduction in corticosteroid dosage, and inflammation usually requiring increased corticosteroid dosage creates a dilemma such that worsening visual acuity should be treated paradoxically with reduction in corticosteroid dosage. A 34-year-old male patient with history of juvenile idiopathic arthritis and uveitis complained of blurred vision in the left eye for 3 days, which was 3 weeks after increasing prednisone dose to 10 mg daily. He was functionally blind in the right eye due to band keratopathy. Vision was 20/25+2 in the left eye. The fundus examination revealed CSR, confirmed by ocular coherence tomography (OCT; Figure 1) and fluorescein … Address correspondence to Dr. S.R Gupta, 3375 SW Terwilliger Blvd., Portland, Oregon 97239; E-mail: seema\_r\_gupta{at}yahoo.com