OPERACIÓN DEL SISTEMA

Henoch schonlein purpura nephitis: initial risk factor and outcomes in a tertiary center of Latin America

Izabel M. Buscatti, Beatriz B. Casella, Nadia E. Aikawa, Andrea Watanabe, Sylvia C. Fahrat, Lucia M. Campos, Clovis A. Silva

Pediatric Rheumatology Division, CHILDREN’S INSTITUTE, HOSPITAL DAS CLINICAS HCFMUSP, FACULDADE DE MEDICINA, UNIVERSIDADE DE SAO PAULO, São Paulo, Brazil

Correspondence:Izabel M. Buscatti

Pediatric Rheumatology2017,15(Suppl 2):P46

Introduction:European League Against Rheumatism/Paediatric Rheuma- tology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) proposed validated classification criteria for Henoch-Schönlein purpura (HSP). However, there are rare studies report- ing initial risk factors to HSP nephritis (HSPN) and outcomes using these new criteria. In addition, these studies generally included small samples, and none of them were evaluated in Latin American population.

Objectives: To evaluate risk factors associated with HSPN and out- comes in children and adolescents of a tertiary center in Latin America.

Methods:Two hundred ninety six patients with HSP were retrospect- ively assessed by demographic data, and initial clinical manifesta- tions, laboratory exams and treatments evaluating in the first three months of disease. All of them fulfilled validated EULAR/PRINTO/PRES criteria for HSP. They were also divided in two groups: with and with- out nephritis. Additional, persistent non-nephrotic proteinuria, neph- rotic proteinuria and renal insufficiency were also evaluated at 1, 5, 10 and 15 years after diagnosis.

Results:Nephritis was detected in 139/296 (47%) at first 3 months. The median of age at diagnosis was significantly higher in HSPN patients compared to those without this complication [6.6(1.5-17.7) vs. 5.7(0.9- 13.5) years, p = 0.022]. The frequencies of persistent purpura (31% vs. 10%, p < 0.0001), recurrent abdominal pain (16% vs. 7%, p = 0.011), gastrointestinal bleeding (25% vs. 10%, p < 0.0001) and corticosteroid use (54% vs. 41%, p = 0.023) were significantly higher in the former group. In the multivariate analysis, logistic regression demonstrated that the independent variables that predicted HSNP were persistent purpura (OR = 3.601; 95%CI 1.605-8.079; p = 0.002) and gastrointestinal bleeding (OR = 2.991; 95%CI 1.245-7.183; p = 0.014). Of 139 HSPN, further analysis revealed that persistent non-nephrotic proteinuria, nephrotic proteinuria and renal insufficiency occurred during follow-up: at 1 year [46/88 (52%), 1/88 (1%) and 2/88 (2%)], at 5 year [25/47 (53%), 1/47 (2%) and 1/47 (2%)], at 10 year [9/20 (45%), 1/20 (5%) and 1/20 (5%)] and at 15 years [1/6 (17%), 0/6 (0%) and 0/6 (0%)]. In addition, in patients without HSPN at disease onset: 29/118 (25%) had persistent non-nephrotic proteinuria and/or hematuria only at 1 year of follow-up, 19/61 (31%) at 5 years, 6/ 17 (35%) at 10 years and 4/6 (67%) at 15 years of follow-up.

Conclusion: Persistent purpura and gastrointestinal bleeding were initial predictors for HSPN. Persistent non-nephrotic proteinuria and/ or hematuria may occur during the disease follow-up, even in pa- tients without previous HSPN, and rigorous monitoring of renal in- volvement should be performed.

Disclosure of Interest: I. Buscatti: None Declared, B. Casella: None De- clared, N. Aikawa: None Declared, A. Watanabe: None Declared, S. Fahrat: None Declared, L. Campos: None Declared, C. Silva Grant/Re- search Support from: Conselho Nacional de Desenvolvimento Científ- ico e Tecnológico (CNPq 472155/2012-1)

P47

Toward the development of a new radiographic score for diagnosis and monitoring of temporomandibular joint disease in children with juvenile idiopathic arthritis

Gabriella Giancane, Giacomo Chiappe, Fiammetta Sertorio, Veronica Incarbone, Alessandro Consolaro, Nicola Laffi, Gian Michele Magnano, Angelo Ravelli

Istituto Giannina Gaslini, Genoa, Italy

Correspondence:Gabriella Giancane

Pediatric Rheumatology2017,15(Suppl 2):P47

Introduction:Arthritis of the temporo-mandibular joint (TMJ) is often responsible for severe osteo-articular damage in patients with juvenile idiopathic arthritis (JIA). Early diagnosis of TMJ involvement remains dif- ficult, due to the lack of reliable clinical or imaging parameters. How- ever, magnetic resonance imaging (MRI) is regarded as the most sensitive tool for the detection of TMJ involvement in JIA.

Objectives:To develop and validate a MRI score for early detection of TMJ disease activity and damage in children with JIA.

Methods:After a review of the most recent literature on MRI of the TMJ in JIA and based on their experience, three specialists in different fields (a rheumatologist, a dentist and a radiologist) devised the score for the assessment of activity and damage of TMJ shown in Table18. The score consists of 3 parameters of arthritis activity (joint effusion, contrast en- hancement (CE) and bone edema), and 4 parameters of joint damage (erosion/irregularities of the mandibular condyle, disc abnormalities, flat- tening of the condyle, mandibular asymmetry). The activity score ranges from 0 to 6 and the damage score from 0 to 9. After a series of training sessions aimed to reach consensus on the definition of the score fea- tures, each specialist assigned independently the MRI score to an unse- lected sample of TMJ MRIs of JIA patients followed at our center. The inter- and intra-observer reliability were calculated through the weighted kappa. The CE parameter was also compared with a recently validated score calculated on a specific region of interest (ROI) of the TMJ MRI in JIA patients1. Agreement was considered acceptable for a k value > 0.60.

Results: A total of 35 TMJ MRIs performed between December 2013 and December 2016 were evaluated. The analysis of the absolute agreement in score assignment among specialists revealed substantial agreement, with greater concordance between the dentist and the radiologist. The rheumatologist tended to assign lower scores. Among disease activity parameters, the lowest agreement was seen for bone edema, whereas the most challenging joint damage parameter was disc abnormalities. There was a moderate agreement between the CE recorded by the study assessors and that obtained through the ROI cal- culation, especially for the rheumatologist and the dentist.

Conclusion:We have developed a new simple and feasible MRI score for the detection and quantification of disease activity and damage of the TMJ in children with JIA. Although the overall score proved re- liable across different specialists, further work is needed to increase concordance for assessment of bone edema and disc abnormalities.

Reference: 1. Resnick CM et al. Quantifying Temporomandibular Joint Synovitis in Children With Juvenile Idiopathic Arthritis. Arthritis Care Res (Hoboken). 2016;68:1795-1802.

Disclosure of Interest:None Declared

Table 18 (Abstract P47).MRI score (CE: contrast enhancement)

TMJ ACTIVITY PARAMETERS

Right Left Total

score Joint effusion YES□NO□ YES□NO□ 0-2 Contrast enhancement (CE) YES□NO□ YES□NO□ 0-2 Bone edema/bone marrow CE YES□NO□ YES□NO□ 0-2 Total activity score 0-3 0-3 0-6 TMJ DAMAGE PARAMETERS

Erosions/irregularities of the condyle

YES□NO□ YES□NO□ 0-2 Disc abnormalities YES□NO□ YES□NO□ 0-2 Flattening of condyle MODERATE/SEVERE

□MILD□ NO□ MODERATE/SEVERE □MILD□ NO□ 0-4

Mandibular asimmetry YES□NO□

IF YES: RIGHT <□LEFT <□ 0-1

P48

Determining disease activity in CIA by optical imaging of phagocyte migration

Sandra Gran1, Lisa Honold2, Olesja Fehler1, Stefanie Zenker1, Sven Hermann2, Michael Schäfers2, Thomas Vogl1, Johannes Roth1 1_{Institute of Immunology, Münster, Germany;}2_{European Institute for} Molecular Imaging, Münster, Germany

Correspondence:Sandra Gran

Pediatric Rheumatology2017,15(Suppl 2):P48

Introduction:Phagocyte recruitment and migration to the site of in- flammation are key events in the early phase of inflammation. They are indispensable for pathogen elimination, tissue repair and restor- ation of tissue homeostasis. However, dysregulated phagocyte infil- tration and a subsequently overwhelming immune response can also cause severe inflammatory disorders. Therefore, targeting and modu- lation of phagocyte infiltration represents a promising new approach to fight inflammatory disorders and diseases, such as rheumatoid arthritis. Furthermore, non-invasive tracking of phagocyte migration to the site of inflammation could extend both scientific knowledge as well as the repertoire of diagnostic strategies in clinical use.

Objectives:Within this study we aimed to establish a Fluorescence reflectance imaging (FRI) based system to visualize and analyze mi- gration properties of different cell populations in inflammatory dis- ease models, like experimental arthritis,in vivo.

Methods: Immortalized murine myeloid progenitor ER-HoxB8 cells were differentiated to neutrophils or monocytes (Wang et al., 2006). Differentiated cells were labeledin vitrowith the membrane-selective fluorescent dyes DIR (Eisenblätter et al., 2009) or DID, respectively. Viability and functionality of labeled cells were confirmed byin vitro

assays. In several mouse models - particularly in a collagen induced arthritis (CIA) mouse model - we investigated the ability and specific properties of different cell populations to migrate to sites of inflam- mation in vivo via fluorescence reflectance imaging (FRI). Using CRISPR-Cas9 technology we introduced targeted gene deletions for main adhesion molecules.

Results:ER-HoxB8 monocytes and neutrophils could effectively be la- beled with DIR or DID.In vitro assays confirmed that viability and functionality of ER-HoxB8 cells was not affected by cell labeling. Sub- sequentin vivoimaging experiments allowed the visualization of mi- grated labeled phagocytes in different murine disease models, thereby cells could be detected at sites of inflammation with high sensitivity and specificity. In a CIA mouse model the amount of immi- grated cells could even be associated closely to disease score and disease severity. Thus, the detection of immigration of labeled cells might also give hints about new inflammatory spots that are about to settle up before they can be detected macroscopically. Further- more, differential cell labeling allowed direct quantitative comparison of differences in migration rates of wildtype and CD18 or CD49d knockout cellsin vivo.

Conclusion:Specific and distinguishable labeling of diverse cell types allows in vivo tracking and subsequent quantification of migrated cells within the same animal. Targeted gene deletion allows analysis of molecular mechanisms relevant for leukocyte recruitment during different stages of arthritis. Correlation of the amount of immigrated cells to disease severity offers new opportunities to non-invasively detect and monitor inflammatory sitesin vivo.

Disclosure of Interest:None Declared

P49

Juvenile Sjögrens syndrome (JSS): comparing glandular ultrasound in primary and secondary JSS

Johannes-Peter Haas, Manuela Krumrey-Langkammerer

German Center for pediatric and adolescent rheumatology, Garmisch- Partenkirchen, Germany

Correspondence:Manuela Krumrey-Langkammerer

Pediatric Rheumatology2017,15(Suppl 2):P49

Introduction:Diagnosis of juvenile Sjögrens syndrome (jSS) although rare seems to be underestimated in pediatric patients. Especially in

patients with undifferenciated mixed connective tissue disease (MCTD) secondary jSS is frequently present but missed in the diag- nostic work-up.

Objectives:Our intend was to characterize distinct, ultrasonographic (US) findings in a cohort of primary- and secondary jSS patients, the latter with a focus on MCTD and attribute these findings to US scores defined in adult SS.

Methods:A single-center study collected data from clinical charts of jSS-patients admitted to the GCPAR. According the EULAR/ACR cri- teria 8 patients with primary jSS, 8 patients with secondary jSS and MCTD and 9 patients with secondary jSS and other collagenoses were included. All ultrasonographic (US) findings were performed by two experienced investigators between 5/2014 and 3/2017 using a GE logic 8 system. Different scoring systems from the literature were compared for accuracy for gland echostructural abnormalities.

Results:A total of 25 patients (22 females, 3 males) with a mean age of 15,8 years have been included. All 9 pjSS patients had sicca symp- toms, this was present in only 56,3% of sjSS. Swelling or pain in major salivary glands occurred in 7 of 9 pjSS but only 5 of 16 sjSS (in- cluding MCTD). US scoring of salivary glands according to Hocevar [1] which had been proven to be accurate in jSS as well [2] showed a mean score of 26 in pjSS and 18,28 in sjSS patients. We could not correlate US score (Hocevar) with the duration of the disease (Pear- sons r =0.197) (Table19).

Conclusion:In patients with pjSS and sjSS salivary gland ultrasound is a helpful, first-line tool not only to detect salivary gland involve- ment but to score the severity of inflammation as well. As sjSS is sup- posed to be underestimated in collagenoses and MCTD, screening of salivary gland by US is usefull even in patients without sicca- symptoms to identified typical, inhomogeneous parchenchymal ap- pearance with hypoechoic lesions suggestive for jSS.

References:

1. Hocevar A. et al;. Rheumatology 2005; Vol 44; pp 768

2. Krumrey-Langkammerer M. et al. 2015: Ultraschall in Med 2015; 36 - A349 Disclosure of Interest:None Declared

P50

Toward the achievement of an agreement between clinical and ultrasound assessment of the ankle region

Stefano Lanni1_{, Alessandra Alongi}1_{, Adele Civino}2_{, Alessandro} Consolaro1,3, Giovanni Filocamo4, Angelo Ravelli1,3

1_{IRCCS Istituto Giannina Gaslini, Genova, Italy;}2_{Pia Fondazione di Culto e} Religione Card. G. Panico, Tricase, Italy;3Università degli Studi di Genova, Genova, Italy;4_{Fondazione IRCCS Ca' Granda Ospedale Maggiore} Policlinico, Milano, Italy

Correspondence:Stefano Lanni

Pediatric Rheumatology2017,15(Suppl 2):P50

Introduction:The ankle is a complex anatomical structure owing to the multiple joint recesses and surrounding tendons. The clinical examination of this joint is a challenging task even for the expert physician. Ultrasound (US) is becoming a useful adjunctive tool to clinical evaluation for the assessment of children with juvenile idio- pathic arthritis (JIA). Disagreement between clinical and US

Table 19 (Abstract P49).Salivary gland US-scores according Hocevar´s classification pjSS (n = 9) sjSS other (n = 8) sjSS in MCTD (n = 8) All (n = 25) Decrease in echogenity 3 (33,3%) 3 (37,5%) 2 (25%) 18 (72%) Inhomogenous parenchyma 9 (100%) 7 (87,5%) 6 (75%) 22 (88%) Hypoechoic areas 9 (100%) 8 (100%) 7 (87,5%) 24 (96%) Hyperechoic reflexes 7 (77,7%) 6 (75%) 6 (75%) 19 (76%) Disturbed border 4 (44,4%) 2 (25%) 0 6 (24%)

examinations in the assessment of the ankle region has been shown in some studies.

Objectives:The aims of the study were: 1) to assess the frequency of clinical signs of articular and periarticular involvement and of US ab- normalities in the different joint recesses and tendon compartments of the ankle region; 2) to investigate the correlation between clinical signs of articular involvement and US abnormalities in the different joint recesses of the ankle region.

Methods:Twenty-seven ankles of 19 patients with JIA with a clinical suspicion of disease involvement were included in the study. At the same consultation, the ankles were scanned by a physician with ex- perience in the US assessment of children with JIA, who was blinded to clinical findings. Clinical and US evaluations focused on tibiotalar and subtalar joints, the tarsal area and on tendon compartments. For each of the joint recesses the presence of swelling, pain on motion and restricted motion and the detection of joint effusion (JE), syn- ovial hypertrophy (SH) and power Doppler (PD) signal inside the area of SH were recorded on clinical and on US evaluation, respectively. US abnormalities were graded on a 4-point semiquantitative scale. Correlation between clinical signs of articular involvement and US abnormalities in the different joint recesses of the ankle region was estimated using the Kendall’s tau (τ) coefficient.

Results:Overall, on clinical assessment swelling was found more fre- quently in the tibiotalar joint (52%), whereas both pain and restricted motion were documented more commonly in the subtalar joint (44% and 41%, respectively). On US assessment JE and SH were detected more frequently in the tibiotalar joint (56% and 67%, respectively); PD signal was displayed more commonly in the subtalar joint (44%). Among tendon compartments, both clinical and US assessments doc- umented a more frequent involvement of flexor tendons (11% and 52%, respectively). Correlation was found between presence of pain or restricted motion on clinical evaluation and detection of PD signal on US in the tibiotalar joint (τ= 0,58, p = 0,002 andτ= 0,57, p = 0,002, respectively). In the tarsal area correlation was found between pres- ence of restricted motion on clinical assessment and detection of PD signal on US (τ= 0,43, p = 0,024) and between presence of pain on clinical assessment and detection of SH on US (τ= 0,41, p = 0,033). No correlation was found in the subtalar joint between any clinical sign of articular involvement and US abnormalities.

Conclusion: Tibiotalar and subtalar joints are more commonly af- fected than the tarsal area on both clinical and US assessment in case of ankle involvement in JIA. Flexor tendons are more frequently inflamed than the anterior and lateral tendon compartments. On clin- ical examination, pain or restricted motion, but not swelling, in the tibiotalar joint and in the tarsal area seem to correlate with the pres- ence of synovitis on US in these joint recesses of the ankle region. The assessment of the subtalar joint remains a challenging task for the physician without the use of US.

Disclosure of Interest:None Declared

P51

Kawasaki disease: initial echocardiogram predicts subsequent coronary disease and immunoglobuline resistance

Dima Chbeir1_{, Jean Gaschinard}1_{, Ronan Bonnefoy}1_{, Constance Beyler}2_, Isabelle Melki1, Albert Faye1, Ulrich Meinzer1

1_{Pédiatrie générale, maladies infectieuses et médecine interne, Paris,} France2Service de cardiologie pédiatrique, Hôpital Robert Debré, Paris, France

Correspondence:Ulrich Meinzer

Pediatric Rheumatology2017,15(Suppl 2):P51

Introduction:Kawasaki disease (KD) is an acute febrile systemic vas- culitis that affects blood vessels of small and medium calibre. With the availability of intensified treatments for most severe patients, it is crucial to early identify patients at high risk for coronary artery aneu- rysms (CAA). However, the available risk scoring systems from Japan have not been validated in European populations. There is little data concerning the link between initial echocardiogram findings and car- diac prognosis.

Objectives:To investigate the first echocardiogram can predict resist- ance to conventional therapy and/or subsequent development of CAA

Methods: We retrospectively analysed demographic, clinical, bio- logical, echocardiographic and therapeutic data from children diag- nosed with KD between 2006 and 2016 at the Robert-Debré University Hospital, Paris, France.

Results:A total of 157 children with KD were included. Initial echo- cardiogram was performed after a median of 6 days of fever and was abnormal in 48 cases (31%). The initial presence of any echocardio- graphic abnormality was strongly associated with resistance to intra- venous immunoglobulin (p = 0.005) and development of coronary artery lesions within the first six weeks of disease (p = 0.01). All pa- tients (n = 7) with persistent coronary abnormalities at one year already had an abnormal initial echocardiogram. In contrast, severity scores had low sensitivity (24-33%) and low specificity (72-81%) to predict immunoglobulin resistance or cardiac involvement.

Conclusion:Abnormalities in the initial echocardiogram can be used to early identify patients with severe Kawasaki disease in populations with mixed ethnic backgrounds.

Disclosure of Interest:None Declared

P52

Childhood-onset Takayasu arteritis: a referral center experience in Turkey

Sezgin Sahin1_{, Duhan Hopurcuoglu}1_{, Sule Bektas}1_{, Ezgi Belhan}1_{, Amra} Adrovic1, Kenan Barut1, Nur Canpolat2, Salim Calıskan2, Lale Sever2, Ozgur Kasapcopur1

1

Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey2_{Pediatric Nephrology, Istanbul University, Cerrahpasa} Medical School, Istanbul, Turkey

Correspondence:Sezgin Sahin

Pediatric Rheumatology2017,15(Suppl 2):P52

Introduction:Takayasu arteritis is a chronic granulomatous vasculitis that effects primarily aorta and its main branches and rarely seen be- fore the age of 18. Delay in diagnosis and treatment is a common feature due to the nonspecific systemic features. There is limited data on clinical features and long-term outcome of Takayasu arteritis. Also, there is need to evaluate efficacy of current therapies, since the spectrum of treatment options have been increased compared to the past.

Objectives:We aimed to report our referral center experience on the clinical features and treatment options of Takayasu arteritis. In addition, we have evaluated the correlation of various activity and damage scores with eachother

Methods:We performed a retrospective chart review for Takayasu ar- teritis between October 2002 and October 2017 at our tertiary refer- ral pediatric rheumatology center. In addition to the current cases that are followed-up, we have comprehensively searched the hospital database for the ICD-9 code 446.7 and ICD-10 code M31.4 to identify Takayasu arteritis patients.

Results: Overall, 16 patients (12 female) have been diagnosed with Takayasu arteritis in last 15 years. Mean age of the patients at disease onset and diagnosis were 10.9 ± 4.8 years and 11.5 ± 4.7 years, re- spectively. While the median duration from the onset of first symp- tom until the diagnosis was 2.4 months (range 0.1-65 months), the mean disease duration was 6.1 ± 5.9 years. The most frequent mani-