P080 Acroosteolysis: a rare complication or a co-occurrence with Juvenile Idiopathic Arthritis?

Background Acroosteolysis refers to a destructive process involving the distal phalanges of the fingers and toes that may extend to metacarpals or metatarsals. Rarely idiopathic, the diagnosis of primary acroosteolysis requires ruling out other causes. Juvenile idiopathic arthritis is an exceptional aetiology of acroosteolysis occurring mainly in psoriatic arthritis. Here by a case of juvenile idiopathic arthritis associated with acroosteolysis of the toes. Methods A 13-year-old girl with no past medical history, presented to our department of rheumatology with oligoarthritis affecting both wrists and knees. She had no familiar history of psoriasis nor rheumatic diseases. She described a dull ache and recurring swelling of knees evolving for >6 years associated with a macular rash of the chest without fever. On examination, the knees were swollen with a limited range of motion of < 90°. Examination of the spine and sacroiliac joints was unremarkable. There was no deformity, no dysmorphic syndrome nor ligamentous hyper laxity. The mucocutaneous examination was normal. Similarly, there was no hepatosplenomegaly or swollen lymph nodes. Laboratory investigations showed high acute phase reactants and normal blood count. Rheumatoid factor, anti-cyclic citrullinated peptide antibodies and antinuclear antibodies were also negative. Besides, she was negative for HLAB-27. Ophthalmic examination did not show any sequelae of uveitis. Results Plain radiograph of the feet revealed bone resorption of the second and fifth distal phalanges without signs of reconstruction. Other secondary causes of acroosteolysis were ruled out. The diagnosis of oligoarticular juvenile idiopathic arthritis was made. In view of the involvement of the distal phalanges, the phenotype of psoriatic arthritis was probable. The patient was initially treated with non-steroidal anti-inflammatory drugs as well as intraarticular injections of corticosteroids in knees. As the flares persisted, she was put on Methotrexate at a dosage of 15 mg per week with marked clinical improvement. Conclusion Our case illustrates a possible occurrence of acroosteolysis of the feet in the field of an active juvenile idiopathic arthritis. It is important to rule out other causes and make a rapid diagnosis in order to ensure appropriate management decisions.

Fifteen-minute guide to managing oligoarticular juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease affecting children and young people today. However, it is not a single disease entity, but an umbrella term that gathers together a heterogeneous collection of complex, chronic inflammatory conditions with oligoarticular JIA the most common form in both Europe and North America. Due to its relative rarity in daily practice and potential to mimic other conditions, oligoarticular JIA can present a diagnostic and management challenge to healthcare professionals in both primary care and general paediatrics. The aim of this article is to provide a summary of the key aspects of diagnosis, investigation and management of this condition, with the hopes of building clinicians’ confidence when facing a possible case of oligoarticular JIA.

A Case Report on Hypervitaminosis D Mediated Juvenile Idiopathic Arthritis

Juvenile Idiopathic Arthritis (JIA) may be defined as prevailing persistent rheumatic malady of unknown etiology in childhood and predominantly presents with peripheral arthritis. Oligoarticular Juvenile Idiopathic Arthritis (OJIA) was prevailing betwixt young female patients which consistently accompanied by anti-nuclear antibodies incontrovertibility and anterior uveitis. Disease complications differ from maturation retardation, osteoporosis and bone deformities. Due to severe Macrophage Activation System (MAS) leads to multi-organ insufficiency and loss of function. The primary goals of treatment are to distribute normal joint function, to perpetuate normal growth and to thwart long-term joint damage and retain normal body homeostasis. Key words: Juvenile Idiopathic Arthritis, Vitamin D Toxicity, Hypercalciuria, Hypervitaminosis.

AB0003 HLA-ALLELES IN SUSCEPTIBILITY TO SYSTEMIC AND OLIGOARTICULAR JUVENILE IDIOPATHIC ARTHRITIS IN THE BELARUSIAN POPULATION

Background:Juvenile idiopathic arthritis (JIA) is a complex trait, the most common rheumatic disease in children. Considering clinical heterogeneity of the disease, the genetic background of particular JIA subtypes may also vary significantly.Objectives:This work was aimed to reveal characteristic patterns of HLA associations within 11 loci for two clinically different forms of JIA in the Belarusian population.Methods:24 patients diagnosed with systemic JIA, 24 patients with oligoarticular JIA and 24 healthy controls were included into the study. The JIA patients were divided into subgroups according to IIAR classification criteria. High-throughput HLA typing was performed using TruSight HLA v2 Sequencing Panel (Illumina) on MiSeq system. Sample analysis was performed using Assign TruSight HLA v2.0 software.Results:DQA1*05:01:01 and DQB1*02:01:01 alleles showed protective effect against both systemic (p = 0.007; OR=0.08; 95% CI=[0.009–0.65] and p = 0.01; OR=0.09; 95% CI=[0.01–0.83]) and oliarticular JIA (p = 0.026; OR=0.16; 95% CI=[0.03–0.79] and p = 0.046; OR=0.2; 95% CI=[0.04–1.00], while for DRB1*03:01 negative association was revealed only for systemic JIA (p = 0.03; OR=0.11; 95% CI=[0.01–0.88]). At the same time the DQB1*04:02:01 (p=0.026; OR=5.88; 95% CI=[1.20–28.72]) and DRB1*08:01:01 (p=0.07; OR=3.94; 95% CI=[1.01–15.39]) allele frequencies were significantly higher in patients with oligoarthritis but not systemic JIA when compared with controls.Conclusion:High-throughput HLA typing revealed distinct HLA-alleles associated with different JIA subtypes in the Belarusian population as well as some alleles protective both for oligoartricular and systemic JIA.Disclosure of Interests:None declared

Synovial fluid neutrophils in oligoarticular juvenile idiopathic arthritis have an altered phenotype and impaired effector functions

Background Neutrophils are the most prevalent immune cells in the synovial fluid in inflamed joints of children with oligoarticular juvenile idiopathic arthritis (JIA). Despite this, little is known about neutrophil function at the site of inflammation in JIA and how local neutrophils contribute to disease pathogenesis. This study aimed to characterize the phenotype and function of synovial fluid neutrophils in oligoarticular JIA. Methods Neutrophils obtained from paired blood and synovial fluid from patients with active oligoarticular JIA were investigated phenotypically (n = 17) and functionally (phagocytosis and oxidative burst, n = 13) by flow cytometry. In a subset of patients (n = 6), blood samples were also obtained during inactive disease at a follow-up visit. The presence of CD206-expressing neutrophils was investigated in synovial biopsies from four patients by immunofluorescence. Results Neutrophils in synovial fluid had an activated phenotype, characterized by increased CD66b and CD11b levels, and most neutrophils had a CD16hi CD62Llowaged phenotype. A large proportion of the synovial fluid neutrophils expressed CD206, a mannose receptor not commonly expressed by neutrophils but by monocytes, macrophages, and dendritic cells. CD206-expressing neutrophils were also found in synovial tissue biopsies. The synovial fluid neutrophil phenotype was not dependent on transmigration alone. Functionally, synovial fluid neutrophils had reduced phagocytic capacity and a trend towards impaired oxidative burst compared to blood neutrophils. In addition, the effector functions of the synovial fluid neutrophils correlated negatively with the proportion of CD206+ neutrophils. Conclusions Neutrophils in the inflamed joint in oligoarticular JIA were altered, both regarding phenotype and function. Neutrophils in the synovial fluid were activated, had an aged phenotype, had gained monocyte-like features, and had impaired phagocytic capacity. The impairment in phagocytosis and oxidative burst was associated with the phenotype shift. We speculate that these neutrophil alterations might play a role in the sustained joint inflammation seen in JIA.

Outcome of children with oligoarticular juvenile idiopathic arthritis compared to polyarthritis on methotrexate- data of the German BIKER registry

Background Oligoarticular juvenile idiopathic arthritis (oligoJIA) is the most commonly diagnosed category of chronic arthritis in children. Nevertheless, there are no evidence- based guidelines for its treatment, in particular for the use of methotrexate (MTX). The primary objective of this analysis is to evaluate the outcomes in patients with persistent oligoJIA compared to those with extended oligoJIA and rheumatoid factor (RF) negative polyarthritis treated with methotrexate. Methods Patients with persistent or extended oligoJIA or RF negative PA recorded in the Biologics in Pediatric Rheumatology Registry (BiKeR), receiving methotrexate for the first time were included in the analyses. Efficacy was determined using the Juvenile Arthritis Disease Activity Score 10 (JADAS 10). Safety assessment included the documentation of adverse and serious adverse events. Results From 2005 through 2011, 1056 patients were included: 370 patients with persistent oligoJIA, 221 patients with extended oligoJIA and 467 patients with RF negative PA. Therapeutic efficacy was observed following the start of methotrexate. Over a period of 24 months JADAS-minimal disease activity (JADAS ≤2) was reached in 44% of patients with persistent oligoJIA, 38% with extended oligoJIA, 46% with RF negative PA, JADAS-remission defined as JADAS ≤1 was reached in 33% of patients with persistent oligoJIA, 29% with extended oligoJIA and 35% (RF negative PA). Patients with extended oligoJIA achieved JADAS remission significantly later and received additional biologic disease-modifying drugs significantly more often than patients with persistent oligoJIA or RF negative PA (p < 0.001). Tolerability was comparable. New onset uveitis occurred in 0.3 to 2.2 per 100 patient years. Conclusions Patients with persistent oligoJIA taking methotrexate are at least as likely to enter remission as patients with extended oligo JIA or polyarticular JIA. Patients with extended oligoJIA achieved JADAS remission significantly later. Within 2 years, almost half of the patients with persistent oligoJIA achieved JADAS-minimal disease activity.

Synovial Fluid Neutrophils in Oligoarticular Juvenile Idiopathic Arthritis Have an Altered Phenotype and Impaired Effector Functions

BackgroundNeutrophils are the most prevalent immune cells in synovial fluid in inflamed joints of children with oligoarticular juvenile idiopathic arthritis (JIA). Despite this, little is known about neutrophil function at the site of inflammation in JIA and how local neutrophils contribute to disease pathogenesis. This study aimed to characterize phenotype and function of synovial fluid neutrophils in oligoarticular JIA.Methods Neutrophils obtained from paired blood and synovial fluid from patients with active oligoarticular JIA were investigated phenotypically (n=17) and functionally (phagocytosis and oxidative burst, n=13) by flow cytometry. In a subset of patients (n=6), blood samples were also obtained during inactive disease at a follow-up visit. Presence of CD206-expressing neutrophils was investigated in synovial biopsies from four patients by immunofluorescence. ResultsNeutrophils in synovial fluid had an activated phenotype, characterized by increased CD66b and CD11b levels, and most neutrophils had a CD16hi CD62Llow aged phenotype. A large proportion of the synovial fluid neutrophils expressed CD206, a mannose receptor not commonly expressed by neutrophils but by monocytes, macrophages and dendritic cells. CD206 expressing neutrophils were also found in synovial tissue biopsies. The synovial fluid neutrophil phenotype was not dependent on transmigration alone. Functionally, synovial fluid neutrophils had reduced phagocytic capacity and a trend towards impaired oxidative burst compared to blood neutrophils. In addition, the effector functions of the synovial fluid neutrophils correlated negatively with the proportion of CD206+ neutrophils.ConclusionsNeutrophils in the inflamed joint in oligoarticular JIA were altered, both regarding phenotype and function. Neutrophils in the synovial fluid were activated, had an aged phenotype, had gained monocyte-like features and had impaired phagocytic capacity. The impairment in phagocytosis and oxidative burst was associated to the phenotype shift. We speculate that these neutrophil alterations might play a role in the sustained joint inflammation seen in JIA.