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Background Clinical examination of juvenile idiopathic arthritis (JIA) patients does not always adequately reflect disease activity, whereas quantitative Dynamic Contrast Enhanced (DCE)-MRI based biomarkers extracted from images of multiple joints have been shown to reliably predict the course of the disease 1. Objectives To investigate the association between DCE-MRI measures of inflammation in a single and in multiple joints and the treatment related clinical changes. Methods 18 patients (12 girls, med. age 12.6 years, med. disease 1.2 years) with polyarticular JIA with more than 3 affected joints or intolerance to more than 3 months of MTX were given Etanercept. Their most clinically affected hand was imaged with DCE-MRI (0.2T Esaote C-Scan) at baseline, 3 and 6 months following the treatment. DCE-MRI was analysed using dedicated software package (DYNAMIKA, IAG). Dynamic Enhanced MRI Quantification (DEMRIQ-V) was calculated as the volume of enhancing voxels within Region of Interest placed around a single or multiple MCPJs. DEMRIQ-V was also weighted by the mean of Maximum Enhancement (ME) and Initial Rate of Enhancement (IRE), the parameters corresponding to the height and slope of the signal intensity vs time curves extracted from the enhancing voxels.2 Clinical scores included active joint (AJ) count. Involvement of less than 3 AJ was considered a clinical response. The differences in DEMRIQ-V between the visits were analysed using t-test, assuming p Results In all patients, in clinically unaffected joints, MRI was able to detect subclinical disease, and in all but 3 patients, significant and/or clinically meaningful changes were documented for DEMRIQ-ME. In 4 patients, DEMRIQ-V scores showed corresponding clinical changes whereas all other patients these markers were non-concordant. DEMRIQ-V score was predictive of clinical outcome: - · in 5 patients, improvement of DEMRIQ-V at month 3 predicted response to treatment at month 6; - · in 4 patients, increase or persistence of a high DEMRIQ-V at month 3 predicted non-response to treatment at month 6; - · DEMRIQ-V measured in a single most affected joint was as predictive as when it was measured in all MCPJs. Conclusions We conclude that DCE-MRI’s ability to detect early disease can reliably support clinical examination. Use of DEMRIQ-V and DEMRIQ-ME scores, which either followed clinical response (DEMRIQ-ME) or predicted clinical outcomes at 6 months (DEMRIQ-V) in most patients can support early clinical and research decisions. References [1] Kubassova O, et al. Arthritis Rheumatol2017;69(suppl 10). [2] Kubassova O, et al. Eur J Radiol2010Jun;74(3):e67–72. Acknowledgements Support by Pfizer Disclosure of Interest None declared

Juvenile idiopathic arthritis is the most common disease in pediatric rheumatology. It is characterized by chronically progressive joint destruction. The temporomandibular joints (TMJs) are involved in up to 87% of patients and may take an asymptomatic course in 69% of cases. Other than contrast-enhanced magnetic resonance imaging (MRI), there are no reliable screening symptoms or non-invasive procedures available to diagnose the inflammation in its acute form. The goal of this study was to establish an imaging-based classification system for TMJ erosion via MRI and cone-beam computed tomography (CBCT) in an effort to improve indication-specific treatment approaches and to facilitate the comparison of findings. A total of 46 patients were included. Contrast-enhanced MRI and CBCT images obtained during treatment by pediatric rheumatologists and orthodontists were available from 23 patients with juvenile idiopathic arthritis. We devised a classification system combining the findings of both imaging techniques based on this patient sample in comparison with CBCT findings from an age- and gender-matched group of 23 non-arthritis patients, taking into consideration the available literature and administration of contrast medium. Our cohort of 46 patients comprised 60% female and 40% male patients with a mean age of 14 years, providing a total of 92 TMJs for evaluation. We were able to apply the findings efficiently and conveniently to this classification system with no relevant interobserver differences. Mild structural abnormalities were noted in 21% of TMJs in the control group, whereas 83% of TMJs in the arthritis group exhibited severe anomalies, including cases of extreme destruction. Age and gender did not affect the degree of destruction significantly. This is the first classification system to link CBCT and MRI with the use of contrast medium. Contrast-enhanced MRI is an internationally recognized technique that permits acute inflammation to be unequivocally diagnosed. Although structural erosion of the TMJs in our arthritis group was generally severe and significant, we were surprised to observe some cases that were clinically asymptomatic.

Die Juvenile idiopathische Arthritis (JIA) ist die haufigste rheumatische Erkrankung im Kindesalter. Sie wird in 7 Subtypen unterteilt. Die Langzeitprognose der Erkrankung hat sich in den letzten 20 Jahren aufgrund neuer Therapiemoglichkeiten erheblich verbessert. Das Kiefergelenk ist in den letzten 10 Jahren als eines der am haufigsten betroffenen Gelenke zunehmend in das wissenschaftliche Interesse der Kinderrheumatologie geruckt. Die MRT-Diagnostik ist zurzeit die Untersuchungsmethode der Wahl. Das Ziel dieses Reviews ist ein Update uber die JIA sowie im Speziellen uber die Beteiligung des ­Kiefergelenks, die Diagnostik, Epidemiologie und Behandlung zu geben.

Die juvenile idiopathische Arthritis betrifft als chronische Arthritis in einem hohen Prozentsatz der Patienten die Kiefergelenke. Aufgrund der besonderen Anatomie kann es zu einer rasch eintretenden Zerstorung der Kiefergelenkkondylen kommen mit allen potentiell sich daraus ergebenden Folgen. Eine fruhe Diagnose und eine entsprechend komplexe und interdisziplinar gefuhrte Therapie, denen sich dieser Ubersichtsartikel widmet, sind fur die Verbesserung des Outcomes von besonderer Bedeutung.

To report the safety and diagnostic performance of magnetic resonance (MRI)--guided core biopsy of osseous lesions in children with chronic recurrent multifocal osteomyelitis (CRMO) that were visible on MRI but were occult on radiography and computed tomography (CT).A retrospective analysis of MRI-guided osseous biopsy performed in seven children (four girls and three boys; mean age 13 years (range 11 to 14) with CRMO was performed. Indication for using MRI guidance was visibility of lesions by MRI only. MRI-guided procedures were performed with 0.2-Tesla (Magnetom Concerto; Siemens, Erlangen, Germany; n = 5) or 1.5-T (Magnetom Espree; Siemens; n = 2) open MRI systems. Core needle biopsy was obtained using an MRI-compatible 4-mm drill system. Conscious sedation or general anesthesia was used. Parameters evaluated were lesion visibility, technical success, procedure time, complications and microbiology, cytology, and histopathology findings.Seven of seven (100%) targeted lesions were successfully visualized and sampled. All obtained specimens were sufficient for histopathological analysis. Length of time of the procedures was 77 min (range 64 to 107). No complications occurred. Histopathology showed no evidence of malignancy, which was confirmed at mean follow-up of 50 months (range 28 to 78). Chronic nonspecific inflammation characteristic for CRMO was present in four of seven (58%) patients, and edema with no inflammatory cells was found in three of seven (42%) patients. There was no evidence of infection in any patient.MRI-guided osseous biopsy is a safe and accurate technique for the diagnosis of pediatric CRMO lesions that are visible on MRI only.

ZusammenfassungDie Kiefergelenke sind die wahrscheinlich am häufigsten betroffenen Gelenke bei Kindern mit juveniler idiopathischer Arthritis. Die therapeutischen Konzepte sind komplex und basieren neben der Verabreichung von lokal und systemisch wirkenden Medikamenten auch auf die kieferorthopädische Versorgung. Für die Wahl der Medikamente stellt sich grundsätzlich die Frage nach dem Befall auch anderer Gelenke und somit einer eher systemischen Therapie. Im Gegensatz dazu steht die isolierte Kiefergelenksarthritis, die eher einer lokalen medikamentösen Behandlung unterliegen sollte. Kieferorthopädisch stehen unterschiedliche Behandlungsansätze zur Disposition, mit dem Ziel der Wiederherstellung von Form und Funktion.

Kinder und Jugendliche mit juveniler idiopathischer Arthritis (JIA) haben in bis zu 87% der Falle einen Befall der Kiefergelenke. Fast die Halfte dieser Patienten weisen keine klinischen Symptome auf, sodass die Diagnosestellung mittels kontrastmittelverstarkter Magnetresonanztomographie erfolgt. Die Behandlung grundet sich auf medikamentose, orthodontische und physiotherapeutische Masnahmen, allerdings gibt es zur Wirkung der verschiedenen therapeutischen Ansatze kaum Daten. In den letzten Jahren wurde immer haufiger uber die erfolgreiche Anwendung der bildgebend gesteuerten intraartikularen Steroidtherapie berichtet.

Involvement of the temporomandibular joints in children with juvenile idiopathic arthritis usually leads to destruction of the mandibular condyles with consistent growth disturbances and facial anomalies. Due to its frequently silent course, temporomandibular arthritis tends to be a diagnostic and therapeutic challenge. In addition to drug therapy, orthodontics and physiotherapy are essential to prevent further progression and restore lost temporomandibular function.

Background: Temporomandibular joints (TMJ) are frequently involved in children with juvenile idiopathic arthritis (JIA), and gadolinium-enhanced magnetic resonance imaging (MRI) is the only modality for an early diagnosis. However, only very few data exist on the appearance of contrast-enhanced MRI of normal juvenile TMJ. Purpose: To define the spectrum of normal MRI findings of juvenile TMJ, and to assess a possible overlap with findings typical for active synovitis in JIA. Material and Methods: 96 children (192 TMJ), 51 boys and 45 girls with a median age of 7.8 years (range 3–13 years), underwent a head MRI. The presence of autoimmune disease, including JIA, was excluded via chart history, available laboratory findings, and the absence of known typical pathological MRI changes (degree of synovial enhancement, hyperintense signal on T2-weighted images in the synovia or bone marrow, and morphologic changes of the mandibular condyle) of the TMJ affected by JIA. Results: In 90 (94%) children, the TMJ showed no MRI abnormalities compatible with arthritis. In three children (3%), the only abnormal MRI finding was a small bilateral joint effusion. A further three children (3%) had a mild synovial enhancement seen on both axial and coronal MR planes in one child and only in the axial plane in the other two children. Signal hyperintensity on T2-weighted images and other corresponding characteristics of TMJ inflammation were lacking in all these six patients. Conclusion: The vast majority of juvenile TMJ in non-rheumatic children shows no MRI abnormalities. Exceptions, including a discrete enhancement of the synovial membrane (3%) or small joint effusions (3%), can occur in a minority of patients, but none of them are accompanied by other signs of inflammation or morphological changes of the TMJ.

OBJECTIVE. Our objective was to describe the spectrum of MRI features in patients with deep and generalized morphea.CONCLUSION. Imaging features of morphea are not specific and usually overlap with those of other disorders involving the skin, fascia, and musculature, such as some types of fasciitis, myositis, and so forth. Nevertheless, the imaging features of morphea reflect pathomorphologic changes of this rare disorder and enable a complete assessment of the disease extent, including depth of infiltration and disease activity.

Background In chronic inflammatory conditions, the need for a more objective measurement of disease activity has been identified. Imaging biomarkers as outcome measurements based on the automated quantification of dynamic contrast enhanced magnetic resonance images (DCE-MRI) have been studied in adult patients with rheumatoid arthritis (RA) 1 . In children with juvenile idiopathic arthritis (JIA) similar knowledge is very limited. Objectives To compare treatment related changes of clinical scores in patients with JIA and automated DCE-MRI quantitative parameters analyzed with a dedicated software Dynamika tm also compared to clinical outcomes of the patients. Methods In patients with polyarticular JIA with insufficient (≥3 affected joints) response or intolerance to ≥3 months Methotrexate, Etanercept was started. Six Slice Axial DCE-MRI of the metacarpophaleangeal (MCP) 2–5 joints in the clinically most affected hand was performed at 3 time points: baseline (BL), month 3 and 6 of treatment using a 0.2 Tesla Esaote C-Scan. Clinical scores included active joint (AJ) counts. Clinical response was considered a state of ≤3 AJ. DCE-MRI was analyzed using regions of interest (ROI) covering synovium in slices where MCPs 2–5 were visible. Output parameters included dynamic MRI quantification scores (DEMRIQvol) corresponding to the volume of enhancing voxels within the synovial ROIs alone or multiplied with the mean of the maximum enhancement (ME) or the initial rate of enhancement (IRE). Differences in DEMRIQvol scores between visits were analyzed using t-test (p Results 18 Caucasian patients (12 girls, median age 12,6 years, median disease duration 1,2 years) were included in the study. Two patients discontinued imaging after BL but continued treatment. In all but 3 of the remaining patients statistically significant and/or clinically meaningful changes were documented for DEMRIQvol ME between visits. In 4 patients clinical and DEMRIQvol scores showed corresponded changes but these were non-concordant in all others patients. Based on DEMRIQvol change (irrespective of the clinical scores) the outcome of the patient could be predicted: in 5 patients improvement of DEMRIQvol scores predicted response to treatment (within 2–6 months after last MRI examination) in 4 patients the increase or the persistence of a high DEMRIQvol predicted non-response to treatment in 7 patients increase in DEMRIQvol (after initial decrease) or persistence of a high DEMRIQ vol predicted flare (in 3 of the patients flare occurred after treatment discontinuation) In all patients subclinical disease could be detected on MRI in clinically unaffected joints. Conclusions Dynamika based scores appear to be useful for depicting disease activity in JIA and seem to support clinical examination by detecting subclinical inflammation. More over, in the present study DEMRIQvol scores were predictive for the outcome of the patients and were able to “foresee” response to treatment, flare of disease, non-response to treatment in most patients possibly making DEMRIQvol scores supportive in research and clinical decision taking. References Kubassova O et al; Eur J Radiol. 2010 Jun;74(3):e67–72. Acknowledgements The study was supported by Pfizer. Disclosure of Interest N. Tzaribachev: None declared, R. Hagoug Employee of: Image Analysis Group, P. Louka Employee of: Image Analysis Group, J. Islam Employee of: Image Analysis Group, M. Hinton Employee of: Image Analysis Group, O. Kubassova Employee of: Image Analysis Group, M. Boesen Shareholder of: Image Analysis Group

To test the hypothesis that MR imaging guided triamcinolone acetonide injection into the sacroiliac joints of children with enthesitis-related arthritis is feasible, accurate and safe; and effectively reduces sacroiliac inflammation and disease progression.A retrospective analysis of 14 children (6/14 [43%] female, 8/14 (57%) male; mean age, 13.2 years; range, 6-16 years) who received MR imaging guided sacroiliac joint injections at 0.2 Tesla or 1.5 Tesla for enthesitis-related arthritis and acute sacroilitis refractory to medical therapy was performed. 20 mg triamcinolone acetonide were injected. Assessed were intra-articular drug delivery; image quality, duration, and complications. Success of therapy was defined by change of sacroiliac inflammation. Remission time and erosions were assessed by follow-up MRI (range, 10-22 months).Twenty four procedures resulted in intra-articular injection. Image quality was sufficient. No complications occurred. Procedure time was 40 min. Sedation time was 22 min. Success of therapy was achieved in 11/14 (79%) children. Sacroiliac inflammation decreased significantly (-59%). Median remission time was 13.7 months. No erosions occurred.MR imaging guided steroid injection of the sacroiliac joints is feasible, accurate, and safe and can effectively reduce sacroiliac inflammatory activity and may therefore aid in the prevention of disease progression.

Temporomandibular joint involvement occurs in up to 87% of the children with juvenile idiopathic with half of the patients being asymptomatic. In these cases the only option to diagnose arthritis of the temporomandibular joints is the gadolinium enhanced magnetic resonance imaging. Therapeutic approaches include drugs, orthodontic and physiotherapeutic management, although only scant data on the effectiveness of these treatments are available. Several reports in recent years have pointed to imaging guided intraarticular steroids as efficient treatment.

Involvement of the temporomandibular joints in children with juvenile idiopathic arthritis usually leads to destruction of the mandibular condyles with consistent growth disturbances and facial anomalies. Due to its frequently silent course, temporomandibular arthritis tends to be a diagnostic and therapeutic challenge. In addition to drug therapy, orthodontics and physiotherapy are essential to prevent further progression and restore lost temporomandibular function.

The purpose of our study was to test the hypothesis that real-time MRI-guided, selective injection procedures of the temporomandibular joints are feasible, accurate, and safe when performed on a clinical open-bore 1.5-T MR system.A retrospective analysis of 67 injection procedures of the temporomandibular joints (55% [37/67] were therapeutic injections, 27% [18/67] were diagnostic injections, and 18% [12/67] were arthrocentesis procedures), performed in 31 patients (58% [18/31] female, 42% [13/31] male; mean age, 14 years; age range, 3-34 years), was made. Seven of 38 (18%) subjects had two temporally separate procedures. Determinations of skin entry points, puncture, and injection were performed under real-time MRI. Data were assessed for rate of successful injections, quantitative and qualitative image quality, time requirements, and occurrence of complications.Drug delivery was successful in all procedures. The quality of real-time FLASH 2D MR images was sufficient in all cases. Real-time MRI proved to be helpful to achieve high rates of intraarticular injections. Contrast-to-noise ratios were sufficiently high for good delineation of relevant structures. Average length of time was 25 minutes (range, 16-53 minutes). No major complications occurred.We accept the hypothesis that real-time MRI-guided selective injection procedures of the temporomandibular joints are feasible, accurate, and safe when performed on a clinical open-bore 1.5-T MR system.

Bridging bronchus (BB) is a rare, congenital bronchial anomaly that is frequently associated with congenital cardiac malformations, especially left pulmonary artery sling. It represents an anomalous bronchus to the right originating from the left main bronchus. Discrimination from other bronchial anomalies is important, since BB is frequently associated with bronchial stenoses due to abnormal cartilage rings. This case study describes the findings of bronchoscopy, bronchography and multidetector computed tomography (MDCT) in three patients. Bronchoscopy was helpful in the description of the severity and length of bronchial stenoses. However, it was not possible to establish a diagnosis of BB based on this method in two patients, since it is difficult or even impossible to differentiate the bifurcation from the pseudocarina. It was not possible to establish the correct diagnosis in all patients based on bronchography or MDCT. MDCT was able to depict the relationship of bronchial and vascular structures, which is particularly important in patients with pulmonary artery sling. Multidetector computed tomography is preferable to bronchography as it is less invasive and due to its short acquisition time it can be performed in children with severe respiratory disease. In the current authors' experience, detection of cartilage rings still requires flexible bronchoscopy.

Die chronische Arthritis ist eine der haufigsten rheumatischen Erkrankungen bei Kindern. Sie ist ebenfalls eine der haufigeren chronischen Erkrankungen im Kindesalter und ein wichtiger Grund fur Langzeitbehinderungen. Die chronische Arthritis fasst eine Gruppe von ahnlichen rheumatischen Erkrankungen zusammen. Die verschiedenen Entitaten manifestieren sich generell als Arthritis des appendikularen Skeletts. Klinisch und radiologisch zeigen sich jedoch unterscheidbare Formen. Basierend auf einem oligo- oder polyarthritischen Bild oder einer systemischen Manifestationsform unterscheidet die ILAR-Klassifiktion der JIA sieben Enitaten- und Untergruppen. Weitere Grosen sind der Rheumafaktor, HLA-B27 und die Psoriasis. Die ILAR-Klassifiktion der JIA unterscheidet folgende Entitaten: Oligoarthritis, Polyarthritis (Rheumafaktor positiv und negativ), systemische Arthritis, Psoriasis-Arthritis, Enthesitis-assoziierte Arthritis und die undifferenzierte Arthritis. Die Rolle der radiologischen Diagnostik besteht aus der initialen Diagnose und Klassifikation der JIA, der differenzialdiagnostischen Abklarung, der Beurteilung des Krankheitsverlaufs und Therapiemonitoring sowie der Detektion von Komplikationen. Lernziele: Korrespondierender Autor: Fritz J Eberhard-Karls-Universitat Tubingen, Radiologische Diagnostik, Hoppe-Seyler-Strasse 3, 72076 Tubingen E-Mail: jan.fritz@gmx.net

Background Juvenile idiopathic arthritis is a chronic inflammatory disease with the challenge of bone and cartilage destruction early in life. Assessment of damage in JIA is therefore of pivotal interest for preceding the long-term disease course in patients with JIA. In particular, the polyarticular JIA is considered being a highly destructive disease. Magnetic resonance imaging (MRI) in adult rheumatoid arthritis patients is known to detect erosions already in early stages. Since in JIA the knowledge on the impact of arthritis on bone structure during the course of the disease is still limited, additional studies are needed to dissect the reversible inflammatory changes from the usually irreversible destructive change in the joints of children (1-3) Objectives To test, whether suspect erosions in JIA patients detected by MRI are indeed associated to structural damage to the cortical bone Methods Twelve children with a median age of 15.87±2.66 years, 10 with polyarthritis and two with psoriatic arthritis received MRI and conventional computed tomography (CT) of the same wrist. Mean disease duration was 2.16 years. All patients were treated with NSAIDs and subcutaneous methotrexate. Due to the relatively small patient cohort a descriptive statistical approach was chosen. Results Overall 1248 surfaces were evaluated both in MRI and CT; 27 erosions were detected by MRI and 46 erosions by CT. Erosions predominated in the capital bone, lunar bone and the second metacarpophalangeal joint. The ulnar styloid, the scaphoid, the carpometacarpal joint, and the 2nd, 4th and 5th metacarpophalangeal joints were not affected. Of the 27 erosions detected in MRI, 23 were confirmed as being true bone erosions in the CT while 4 lesions were identified as vessel channels but not erosions. Widths of erosions in the MRI varied from 0.34 to 7.34mm, and depths from 0.98 to 5.35mm. In the CT widths ranged from 0.57 to 4.22 mm, and depths from 0.72 to 5.36mm. Details of sensitivity, specificity as well as positive and negative predictive value (PPV/NPV) see table 1. Conclusions MRI bone erosions in patients with JIA mostly correspond to cortical breaks detected by conventional CT suggesting structural bone damage in patients with JIA. References Chedeville G1, Azouz M, Scuccimarri R. A104: aggressive erosive synovitis in juvenile idiopathic arthritis. Arthritis Rheumatol. 2014 Mar;66 Suppl 11:S140. doi: 10.1002/art.38525. Ording Muller LS1, Boavida P, Avenarius D, et al. MRI of the wrist in juvenile idiopathic arthritis: erosions or normal variants? A prospective case-control study. Pediatr Radiol. 2013 Jul;43(7):785-95. doi: 10.1007/s00247-012-2575-z. Epub 2013 Jan Avenarius DM1, Ording Muller LS, Eldevik P, et al. The paediatric wrist revisited–findings of bony depressions in healthy children on radiographs compared to MRI. Pediatr Radiol. 2012 Jul;42(7):791-8. doi: 10.1007/s00247-012-2354-x. Epub 2012 Mar 20. Disclosure of Interest None declared

SUMMARYCommon variable immunodeficiency (CVID) is a heterogeneous immunodeficiency that is accompanied by granulomatous lesions in 5–10% of cases. Why some patients develop granulomatous disease remains unclear. Here we describe a 12-year-old previously healthy girl who presented with pancytopenia and granulomatous lymphoproliferation subsequent to infection with Toxoplasma gondii. Loosely arranged non-fibrosing granulomas were observed in the liver, lymph nodes and lung, but no Toxoplasma tachyzoites could be demonstrated and polymerase chain reaction (PCR) and culture were negative for Toxoplasma and a wide range of other pathogens. While the patient had a normal peripheral B cell status at presentation, the development of CVID could be observed during the following months, leading to a loss of memory B cells. This was accompanied by an increasingly activated CD4+ T cell compartment and high serum levels of angiotensin-converting enzyme (ACE), tumour necrosis factor (TNF) and sCD25. Steroid therapy reduced pancytopenia, granulomatous lymphoproliferation and cytokine elevations, but did not improve the B cell status. This is the first report of an association of Toxoplasma infection with granulomatous CVID and provides one of the rare examples where the onset of CVID could be documented subsequent to an infectious disease.

Background The TNF Alpha (TNFA) antagonist etanercept (ETN) is approved for the treatment of polyarticular juvenile idiopathic arthritis (JIA) in a dose of 0.8 mg/kg/week. The existing data for TNFA other than ETN suggest a higher efficacy if higher doses are applied. Objectives To evaluate the efficacy and safety of ETN in doses >0.8 mg/kg/week in JIA patients. Methods Data from the German Etanercept BIKER Registry were evaluated. Patients were divided into three groups: high dose (HD) (>0.8 mg/kg/week), low (standard) dose (LD) (≤0.8mg/kg/week) and mixed dose (MD) (switching between (>0.8 and ≤0.8mg/kg/week). Efficacy was measured by the PedACR score 30, 50, 70 after 3, 6 and 12 months of treatment. Safety analysis was performed by counting the number of adverse events (AE) per group and per patient. Last observation carried forward (LOCF), intention to treat (ITT) and per protocol (PP) data processing was applied. Statistically Kruskal-Wallis and χ2-tests were used. Results 547 patients (358 females) with a median age at registry entry of 6.6 years were evaluated: 264 in HD, 109 in the LD and 174 in MD groups. Efficacy: Independently of the data processing method (LOCF, ITT, PP), efficacy was similar irrespective of the dose. Differences between HD, LD and MD groups could not reach statistical significance. Interestingly the number of patients reaching a higher PedACR score increased with the duration of ETN-treatment, e.g. in the ITT analysis 38.9% of patients reached a PedACR 70 at 3 months of treatment vs. 59.6% at 12 months. Safety: A total of 72 AE occurred. Divided by the three treatment groups these were: 40 (15.15%) in the HD, 13 (11.93%) in the LD and 19 (10.92%) in the MD groups. The difference could not reach statistical significance (p=0.40). Conclusions Higher doses of ETN in children with JIA do not seem to add additional benefit to the treatment result. No statistically significant differences could be found between the three treatment groups and therefore in contrast to other TNF Alpha antagonists higher doses shouldn’t be recommended to improve outcome. The duration of treatment with ETN seems to have a positive effect on the response rate to the drug. Disclosure of Interest None Declared

Background Patients with polyarticular juvenile idiopathic arthritis (JIA) may not respond sufficiently to methotrexate (MTX) combined with TNF Alpha antagonists (TNFA). In this cases switching to another TNFA might be beneficial. In case of non-response to the second TNFA it is recommended to change the treatment principle to another biologic. Certolizumab pegol (CZP) consists in a Fab fragment of a pegolated monoclonal TNF Alpha antibody. CZP is the only pegolated (conjugated with polyethylenglycol) TNFA so far. Pegolation is meant to be one of the reasons for improved pharmacokinetic properties leading to site dedicated suppression of the inflammation. Objectives The aim of the retrospective study was to evaluate the efficacy and safety of CZP in children with polyarticular course JIA not responsive to other TNFA. Methods Based on a search of the hospital database patients with polyarticular course JIA treated with CZP and at least one other TNFA (prior to CZP) were identified. The active joint count was documented at week 0, week 6-12 and week 24-36. Efficacy was evaluated by the decrease of the number of active joints over time. For the safety analysis adverse events (AE) were documented. Results 22 patients (4 boys) were identified. The age range at initiation of CZP treatment was 9-17 years. 18 children were treated with 2 TNFA before CZP was introduced. 18 patients received concomitantly subcutaneous MTX. At week 0 all patients had active joints (median 14, min. 3, max. 36). At week 6-12 one patient did not respond. Out of the remaining 21 patients 0 active joints were found in 11; the active joint count has decreased in the remaining 9 (median 2, min. 0, max. 4). At week 24-36 the number of patients with active joints has further decreased to 6 (15 patients had 0 active joints). The remaining 6 patients had a median active joint count of 2 (min. 1, max. 4). In this cohort only one AE occurred – skin reaction to CZP, which disappeared spontaneously. Conclusions Despite the recommendation of switching the treatment principle after non-response to two TNFA in children with JIA, the results of this study point to the capacity of CZP to gain control over the disease in this short-term analysis and therefore CZP might become a treatment option in those cases (the drug is still off-label use). CZP treatment was overall well tolerated. Disclosure of Interest None Declared