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Antineutrophil cytoplasmic antibody (ANCA) may appear in the course of rheumatic diseases (RD) but the kidney involvement is very rare and the prognosis poorly defined.We retrospectively identified patients with RD among 153 patients with ANCA glomerulonephritis (ANCA-GN). Their clinical/histological presentation and outcome were compared with that of primitive ANCA-GN patients (1:4) matched for sex, age, ANCA type and follow-up.Nine patients (5.9%) were included: three had rheumatoid arthritis, two systemic sclerosis, two psoriatic arthritis, one ankylosing spondylitis and one seronegative spondylarthritis. Seven patients were MPO positive, two PR3 positive. ANCA-GN developed 74 months after RD with microscopic haematuria and acute kidney dysfunction in all but two patients. After 68-month follow-up, four patients (44.4%) achieved response to therapy defined as eGFR60/min/1,73 m2 or stable, no microscopic haematuria and negative ANCA. At ANCA-GN diagnosis, serum creatinine and C-reactive protein were significantly lower in RD-ANCA-GN (2.38 vs. 3.34mg/dl, p=0.05 and 2.3mg/dl vs. 7.2mg/dl; p=0.05, respectively) while haemoglobin was higher (12.3g/dl vs. 9.3g/dl p0.01) than in the 36 primitive ANCA-GN patients of control group. At kidney biopsy, focal forms were more frequent in RD patients (44.45% vs. 18.75%, p=0.11). The treatment between the two groups was not significantly different. At last observation, the percentage of patients with ESKD was lower in RD than in controls (11.1%vs. 30.5%; p=0.23).Patients with RD seem to develop ANCA-GN with less severe clinical/histological kidney involvement, and better long-term kidney survival than primitive ANCA-GN. This is probably due to the strict monitoring of RD patients that allows a prompter ANCA-GN diagnosis and treatment.

Highlights • Systemic lupus erythematosus (SLE) associates with infection susceptibility. • COVID-19 is a pandemic infectious disease with high morbidity and mortality. • The impact of COVID-19 in SLE is poorly characterised. • A web-based survey amongst patients with SLE suggest a moderate increase in morbidity due to COVID-19. • Hydroxychloroquine does not seem able to prevent COVID-19 in patients with SLE., Background Systemic lupus erythematosus (SLE) is a chronic disease characterised by autoimmunity and increased susceptibility to infections. COVID-19 is a systemic viral disease currently spreading as a pandemic. Little is known about the impact of COVID-19 in patients with SLE. Objective to acquire information on the impact of COVID-19 in SLE. Methods A 26-item anonymous questionnaire investigating demographics, SLE clinical features, COVID-19 diagnoses and changes in treatments and daily habits was administered to patients with SLE from three referral centres through www.surveymonkey.com over 10 days. Data from the survey were compared to those from published estimates about the general population. Results Four-hundred-seventeen patients responded to the survey. More than 60% of subjects complained of symptoms that are also associated to COVID-19. Fourteen COVID-19 diagnoses (five confirmed by polymerase chain reaction) were reported, in contrast to a 0.73% prevalence of confirmed cases in Lombardy. One hospitalisation was reported. Fever, anosmia, dry cough, a self-reported history of neuropsychiatric SLE and a recent contact with confirmed COVID-19 cases were more strongly associated with COVID-19, as were symptoms and lower compliance to behavioural preventive measures in patients’ contacts. No protective effect was seen in subjects on hydroxychloroquine. Conclusion COVID-19 morbidity might only moderately be increased in most patients with SLE, although limited information can be inferred on more severe cases. Hydroxychloroquine apparently seems not to confer protection to infection per se, although other beneficial roles cannot be excluded. Containment policies and behavioural preventive measures could have a major role in limiting the impact of COVID-19 in patients with SLE.

Interstitial lung disease (ILD) has been described as a possible pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV), mainly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Aim of this cross-sectional Italian national study was to describe demographic, clinical and serological profile of ILD related to MPA and GPA and investigate possible correlations between radiologic patterns of ILD and vasculitis features.We enrolled 95 consecutive patients with AAV-ILD, 56 affected by MPA (58.9%) and 39 by GPA (41.1%).NSIP was the most frequently detected ILD pattern, observed in c-ANCA patients in 60.9% of cases, followed by UIP pattern mainly observed in p-ANCA patients (47.7%, p=0.03). ILD represented the first clinical manifestation, preceding vasculitis diagnosis in 22.1% of cases and, globally, ILD was already detectable at AAV diagnosis in 66.3% of patients. The diagnosis of ILD preceded that of AAV in 85.7% of p-ANCA positive-patients, while only one patient with c-ANCA developed ILD before AAV (p= 0.039). Multivariate analysis confirmed the correlation of UIP pattern with p-ANCA-positivity and a diagnosis of ILD before AAV, also when adjusted for age and sex.Our study confirms that UIP is a frequent pattern of lung disease in AAVILD patients. Our results also suggest that ILD can represent an early complication of AAV but also occur in the course of the disease, suggesting the need of a careful evaluation by both pulmonologist and rheumatologist to achieve an early diagnosis. Further prospective studies are needed to define ILD prevalence and evolution in AAV patients.

Objective: This retrospective cohort study describes the modulation of disease activity during gestation and in the year following delivery as well as maternal and neonatal outcomes in a monocentric cohort of women with juvenile idiopathic arthritis (JIA). Methods: Disease activity was assessed using DAS28-CRP before conception and every 3 months during pregnancy and in the first year postpartum. The risk of complicated pregnancies was measured applying a generalized estimating equation model. Changes in disease activity during gestation and in the first year postpartum were assessed in a linear mixed model for repeated measures. Results: Thirty-one women (49 pregnancies) with persisting JIA and at least one conception were enrolled. Adjusted DAS28-CRP levels remained stable from preconception through the first trimester, but increased significantly in the second and decreased not significantly in the third. In the postpartum, adjusted disease activity peaked at 3 months after delivery, stabilized at 6 months to decrease at 1 year, although not significantly. Preconceptional DAS28-CRP and number of biological drugs predicted disease activity fluctuation during gestation. The number of biological drugs and the length of gestational exposure to biologics significantly predicted pregnancy morbidity. In particular, JIA women had a higher probability of preterm delivery compared with healthy and disease controls. Adjusted for breastfeeding and DAS28-CRP score in the third trimester, postconceptional exposure to biologics was inversely related with disease activity in the postpartum: the longer the patient continued treatment, the lower the probability of experiencing an adverse pregnancy outcome. Conclusion: These data offer novel insights on how treatment affects disease activity during pregnancy and postpartum as well as obstetric outcomes in women with JIA.

The ideal long-term maintenance therapy of Lupus Nephritis (LN) is still a matter of debate. The present study was aimed at comparing the efficacy/safety profile of cyclosporine (CsA), mycophenolate mofetil (MMF) and azathioprine (AZA) in long-term maintenance therapy of LN.We performed a retrospective study of patients with biopsy-proven active LN. After induction therapy, all patients received maintenance therapy with CsA, MMF or AZA based on medical decision. Primary endpoint was complete renal remission (CRR) after 8 years (defined as proteinuria 0.5 g/24 h, eGFR 60 ml/min/1.73 mq); secondary endpoints were: CRR after 1 year, renal and extrarenal flares, progression of chronic kidney disease (CKD stage 3 or above) and side-effects.Out of 106 patients, 34 received CsA, 36 MMF and 36 AZA. Clinical and histological characteristics at start of induction therapy were comparable among groups. At start of maintenance therapy, CsA patients had significantly higher proteinuria (P = 0.004) or nephrotic syndrome (P = 0.024) and significantly lower CRR (23.5% vs 55.5% on MMF and 41.7% on AZA, P = 0.024). At one year, CRR was similar in the three groups (79.4% on CsA, 63.8% on MMF, 58.3% on AZA, P = 0.2). At 8 years, the primary endpoint was achieved by 79.4% of CsA vs 83.3% of MMF and 77.8% of AZA patients (P = 0.83); 24 h proteinuria, serum creatinine, eGFR were similar. CKD stage 3 or above developed in 8.8% of CsA, in 8.3% of MMF and in 8.3% of AZA patients (P = 0.92). Flares-free survival curves and incidence of side-effects were not different.This is the first study comparing CsA, MMF and AZA on long-term LN maintenance therapy. All treatments had similar efficacy in achieving and maintaining CRR, despite more severe baseline clinical features in patients treated with CsA.

Biological agents have radically changed the prognosis of rheumatic patients. Current evidence demonstrates that tight disease control during pregnancy is mandatory to minimize adverse outcome risk. As the new therapeutic tools are pivotal to maintain appropriate disease activity, it is timely to review available evidence about the safety of biologics and small molecules in pregnancy. Areas covered: A comprehensive literature review has been performed, reporting available data about the passage into breast milk, rate of pregnancy loss and fetal malformations, and long-term complications due to in utero exposure to biological agents and small molecules. Expert commentary: Data about the safety of agents against tumor necrosis factor in pregnancy are reassuring. Even rituximab, tocilizumab, belimumab, ustekinumab, secukinumab, and abatacept have not been associated with an increased rate of fetal abnormalities or adverse pregnancy outcome. Experience with small molecules is too small to draw any conclusion. Even if further data are warranted to define the possible long-term effects of in utero biologic exposure on the infant immune system development, it is reasonable to speculate that in the next future the use of biologics during pregnancy will continue to expand, at least when maternal benefit justifies the potential risk to the fetus.

Little is known about the impact of coronavirus disease 2019 (COVID-19) pandemic to the care of patients with systemic lupus erythematosus (SLE) in the long-term. By crossing population data with the results of a web-based survey focused on the timeframes January–April and May–December 2020, we found that among 334/518 responders, 28 had COVID-19 in 2020. Seventeen cases occurred in May–December, in parallel with trends in the general population and loosening of containment policy strength. Age > 40 years (p = 0.026), prednisone escalation (p = 0.008) and infected relatives (p

Background The most effective drug for the maintenance therapy of severe forms of lupus nephritis (LN) is still a matter of debate. Objectives To compare efficacy and safety of cyclosporine (CYA) with mycophenolate mofetil (MMF) and with azathioprine (AZA) in the long-term maintenance therapy of LN. Methods Ninety-six patients (pts) (93 females, mean age 31.98±12.78 years) with SLE and biopsy proven LN (16 pts:class III; 64 pts:class IV; 16 pts:class V ISN/RPS). Fifty-six pts entered this study at diagnosis of LN and 40 during a course of a LN flare. Twenty-five pts (30%) had Glomerular Filtration Rate (eGFR) 60ml/min and proteinuria 60ml/min and proteinuria >0.5/die, no response: eGFR Results At the beginning of maintenance therapy, the mean serum creatinine and eGFR were similar in the 3 groups (0.92±0.26mg/dl, eGFR 109,9±49,5ml/min in CYA, 0.86±0.4mg/dl, eGFR 119±44,6 in MMF, 0.85±0.3mg/dl, eGFR 106,6±43,9ml/min in AZA). Proteinuria was higher in CYA group (CYA:2.03±1.7g/day; MMF:0.77±0.8g/day; AZA:1.2±1.1g/day). At the beginning of maintenance therapy, complete, partial and no response were 26.6%, 60%, and 13.4% in CYA, 53.1%, 43.8% and 3.1% in MMF and 38.2%, 58.8% and 3% in AZA group (Fig 1). At 1 year, after 6 months of maintenance therapy, in CYA group the percentage of pts in complete remission increased to 73% (vs 65.6% in MMF and 40% in AZA), at 5 years it was 80% (vs 83% in AZA and in MMF) and 88% at 10 years vs 70% in MMF and 68% in AZA (Fig 2,3,4). The percentage of non-responsive pts was stable from 1 to 10 years in the CYA group (around 13%), it slightly increased in MMF group (from 3.1 to 13,5%) and in AZA group (from 15 to 24%). During the study, SLE flares occurred in 30% of CYA group, 41% in MMF and 32% of the AZA. The average time from the beginning of the study and the first flare was 3.95±2.76years in CYA, 3.62±1.60 in MMF and 5.9±2.37 in AZA. No side effects were reported in 90% of pts treated with CYA, in 81.3% with MMF and in 85.3% with AZA group. Conclusion This is the first study comparing these 3 drugs as maintenance therapy in the long term. After 10 years of observation, CYA, AZA and MMF have proven to be effective in consolidating and maintaining the remission of LN. Of interest are the results achieved in the CYA group. Despite worse clinical conditions at the beginning of maintenance therapy, CYA allowed a rapid achievement of LN remission in the great majority of pts compared to AZA and MMF. Remission persisted over 10 years of observation. The number and type of flares and of side effects were not different between groups. Reference [1] Moroni G, et al. 2006 Disclosure of Interests Lorenza Maria Argolini: None declared, Elena Elefante: None declared, Francesca Saccon: None declared, Valentina Binda: None declared, Maria Gerosa: None declared, Luigi Sinigaglia Speakers bureau: Yes, I,ve been invited speaker by Amgen, Ely Lilly, UCB, Abbvie, Roche and BMS., Piergiorgio Messa: None declared, Andrea Doria: None declared, Marta Mosca Paid instructor for: GlaxoSmithKline, Lilly, UCB, Gabriella Moroni: None declared

Introduction: Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by vascular thrombosis and/or pregnancy morbidity associated with the persistent presence of antiphospholipid antibodies (aPL) including lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and anti-β2 glycoprotein I antibodies (aβ2GPI).Areas covered: APS is considered as the most common acquired hypercoagulation state of autoimmune origin in children. Unfortunately, data about incidence, prevalence, thrombosis risk and effective treatment in paediatric APS are limited and unmethodical.Expert commentary: This review summarizes recent clinical, laboratory and therapy characterization of paediatric APS and emphasizes the differences between paediatric and adult populations.

Background:the prognosis of Systemic lupus Erythematosus (SLE) patients has significantly improved over time, raising the need for more data about disease activity and damage accrual in the long term.Objectives:to investigate the risk of long term disease activity and to identify viable prognostic markers for disease flares in SLE patients with long standing diseaseMethods:data on SLE patients regularly followed at ASST PINI-CTO, Fondazione Ca’ Granda Policlinico and Ospedale San Raffaele, Milan (Milan Systemic Lupus Erythematosus Consortium, SMiLE, cohort) with disease duration ≥ 20 years, were retrospectively analyzed. Organ involvement as per the British Isles Lupus Assessment Group (BILAG) definitions was recorded along with achievement of clinical and complete remission (CR and CCR: clinical SLEDAI =0, PGA Results:data from 168 patients (table 1) were available for analysis. Remission (CR+CCR) and LLDAS were achieved in 22% and 61% at T15 and 25% and 71% at T20. LLDAS was not associated with a history of involvement in any BILAG domain, but it was inversely associated with treatment with mycophenolate at any time (50 vs 23% treated vs not treated; p=0.02). SDI>0 was found in 49% patients at T15 and in 71% at T20. LLDAS at T15 was associated with lower flare rates in the following five years (HR= 0.395, 95%, CI=0.239-0.653; Figure, left panel; pTable 1.Demographic, laboratory and clinical characteristics of patients with SLECharacteristics(n=168)Demographic characteristicsFemale, n (%)150 (89.3)Age at diagnosis years, median (IQR)24 (18-32)Clinical and serological features during 15 years follow up, n (%)Musculoskeletal140 (83)Mucocutaneous135 (80)Constitutional114 (68)Haematological103 (61)Nephritis81 (48)Cardiopulmonary49 (29)NPSLE31 (18)Positive anti-dsDNA136 (81)Hypocomplementemia136 (81)Positive antiphospholipid73 (44)CR / CCR at T1513 (8) / 23 (14)CR / CCR at T2017 (10) / 25 (15)Conclusion:LLDAS is common in SLE patients with long disease duration although up to 37% of patients with 15-year disease duration may experience a flare during the following 5 years. The flare risk increases with failure to attain LLDAS at T15 and with active serology. Late flares associate with damage accrual.References:[1]Aringer M, Ann Rheum Dis. 2019; Franklin K, Ann Rheum Dis 2019Table 2.therapy during the 20 year follow upTherapy (ever)%Prednisone83Hydroxychloroquine91Mycophenolate mofetil33Azathioprine50Methotrexate23Cyclophosphamide36Fig 1:risk of flare according to disease activityDisclosure of Interests:Maria Gerosa: None declared, Giuseppe Alvise Ramirez: None declared, Chiara Bellocchi: None declared, Lorenza Maria Argolini: None declared, Luca Moroni: None declared, Martina Cornalba: None declared, Nicola Farina: None declared, Lorenzo Dagna Grant/research support from: Abbvie, BMS, Celgene, Janssen, MSD, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, SG, SOBI, Consultant of: Abbvie, Amgen, Biogen, BMS, Celltrion, Novartis, Pfizer, Roche, SG, and SOBI, Roberto Caporali Consultant of: AbbVie; Gilead Sciences, Inc.; Lilly; Merck Sharp & Dohme; Celgene; Bristol-Myers Squibb; Pfizer; UCB, Speakers bureau: Abbvie; Bristol-Myers Squibb; Celgene; Lilly; Gilead Sciences, Inc; MSD; Pfizer; Roche; UCB, Enrica Bozzolo: None declared, Lorenzo Beretta Grant/research support from: Pfizer

Background:IL-10 plays a key role in systemic lupus erythematosus (SLE) pathogenesis, promoting B-cell response. IL10 is mainly secreted by regulatory T-cells, but follicular helper T-cells (TFH), also produce it. We previously identified a subset of CCR6+IL-7R+T-cells in human tonsils providing IL-10-dependent B-cell help. These CCR6+T-cells were able to produce IL-10, inducing IgG production.Objectives:to investigate a possible role of CD4+CCR6+IL7R+T-cells in SLE pathogenesis.Methods:37 patients fulfilling the ACR criteria for SLE have been included. Disease activity was assessed by 2k-SLEDAI. PBMC were analyzed by flow cytometry, using specific lineage markers. CCR6+IL7R+T-cells purified from total PBMC of SLE patients or healthy donors (HD) were co-cultured with autologous CD20+B-cells. IL-10, Il-17, total IgG and anti-dsDNA antibodies titers in patients serum and culture supernatants were assessed by ELISA. Embedded sections of lymph nodes from 8 SLE patients were analyzed by immunofluorescence (IF).Results:IL10 levels were significantly higher in SLE patients (Fig 1A). CD4+CCR6+IL7R+T-cells were significantly increased in SLE, in particular in those with higher disease activity and higher IL10 levels. CD4+CCR6+IL7R+T-cells levels associated with anti-dsDNA positivity. CCR6+IL7R+T-cells of SLE patients induced production of IgG and anti-dsDNA IgG (in anti-dsDNA + patients) from autologous B-cells, providing spontaneous help for autoantibody productionex vivo(Fig 1B-C). The IF study of lymph nodes of SLE patients showed that IL-10-producing CCR6+T-cells were highly abundant and co-localized with B-cells at follicle margins.Fig 1Conclusion:our study revealed a novel population of extra-follicular B-helper T-cells, which produce IL-10 and could play a prominent pathogenic role in SLE. Further studies will clarify if this potentially pathogenic cell population might represent a possible future therapeutic target.References:[1]Facciotti F. J Allergy Clin Immunol. 2016; Geginat J. Semin Immunol. 2019; Tsokos GC. Nat Rev Rheumatol. 2019Tab 1:SLE patients characteristics(n=37)DemographicsFemale/Male, n37/5Age, years, median (IQR)44 (38-49)Disease duration, years, median (IQR)19 (11-26)Lab testsANA86%*anti-dsDNA (%)46% medium/high titre41%Disease activity and clinical manifestations SLEDAI-2K, median (min-max)3.5 (0-24) Moderate/high activity19%Ongoing therapyPrednisone dose mg/day, median (IQR)7,5 mg (2,5 – 20)hydroxychloroquine78%Immunosuppressants87%Fig 2Disclosure of Interests: :Maria Gerosa: None declared, Federica Facciotti: None declared, Paola Larghi: None declared, Roberto Bosotti: None declared, Chiara Vasco: None declared, Nicola Gagliani: None declared, Chiara Cordiglieri: None declared, Elsa Rottoli: None declared, Alessandra Emiliana Penatti: None declared, Lorenza Maria Argolini: None declared, Bhavna Karnani: None declared, Yasushi Kobayashi: None declared, Mauro Bombaci: None declared, Jan Piet Van Hamburg: None declared, Roberta Gualtierotti: None declared, Stefano Gatti: None declared, Sara Torretta: None declared, Lorenzo Pignataro: None declared, Sander W. Tas: None declared, Roberto Caporali Consultant of: AbbVie; Gilead Sciences, Inc.; Lilly; Merck Sharp & Dohme; Celgene; Bristol-Myers Squibb; Pfizer; UCB, Speakers bureau: Abbvie; Bristol-Myers Squibb; Celgene; Lilly; Gilead Sciences, Inc; MSD; Pfizer; Roche; UCB, Sergio Abrignani: None declared, Massimiliano Pagani: None declared, Fabio Grassi: None declared, Pier Luigi Meroni: None declared, Richard Flavell: None declared, Jens Geginat: None declared

Background:neonatal lupus syndrome (NLS) is an acquired disease caused by the trasplacental passage of anti-SSA antibodies. Congenital heart block (CHB) represents the most serious manifestation of NLS. The rate of CHB in Anti-SSA positive pregnant women ranges from 1 to 5% in different studiesObjectives:to retrospectively assess the prevalence of CHB in a cohort of anti-SSA positive pregnant women followed in 3 Italian tertiary centersMethods:pregnancies of anti-SSA positive women attending the pregnancy clinic of ASST Pini CTO/Policlinico Mangiagalli, Rheumatology Division of Spedali Civili, Brescia and Rheumatology Division of Ospedale S Matteo, Pavia from 2009 to 2019 were included. Patients underwent monthly clinical examination. Fetal heart rate was assessed weekly by Doppler ultrasound from 14thto 26thgestational week. On week 14 and 26, a fetal echocardiography was performed. A EKG was performed at birthResults:351 prospectively followed pregnancies in 292 anti-SSA/Ro positive women were included. Table 1 reports diagnosis. None of the prospectively followed pregnancies were complicated by complete CHB. Seven additional patients were referred to our clinics after diagnosis of CHB and were subsequently found to be anti-SSA positive, reporting no symptoms of diseases. Considering the 7 additional pregnancies, the incidence of CHB was 1.9%. We observed 3 neonates (0.8%) with cutaneous NLS and 1 case of transient increase of liver enzymes. In another neonate, a 1thdegree A-V block was found after birth. A complete analysis of maternal and fetal outcome was possible in 244 cases (Table 2) and compared with 3158 unselected healthy controls. Among these 244 cases, 65% were taking hydroxychloroquineTable 1.patients diagnosisn%Sjogren’s Syndrome58`20Systemic lupus erythematosus7626UCTD7425Asymptomatic Ro carriers5619Other2810292100Table 2.maternal and fetal outcomehealthy controls N=3158Anti-SSA/Ro ptsN=244P valuePrevious CHB n (%)2 (0.8)Anti-SSB pos n (%)46 (18.8)aPL pos n (%)49 (20)PregnancyLive births3158241Preeclampsia, n (%)43 (1.1)2 (0.8)nsDeliveryDelivery 401 (12.6) /201 (6)35 (15.6) / 14 (6)ns / nsCesarean Section, n (%)897 (29.3)115 (47.5)Conclusion:none of the patients prospectively followed in our centers before and during pregnancy developed complete CHB. If the 7 cases of anti-SSA positivity diagnosed after CHB detection were included in the analysis, the incidence of CHB was comparable to previous reports. Our data suggest that a strict follow up and proper treatment of anti-SSA positive patients with or without an autoimmune disease before and during pregnancy can reduce the risk of NLS. Further studies are warranted to confirm a possible protective role of anti-rheumatic treatments, including HCQReferences:[1]Fredi M. Front Cardiovasc Med. 2019Disclosure of Interests:Maria Gerosa: None declared, Micaela Fredi: None declared, Laura Andreoli: None declared, Cecilia Chighizola: None declared, Lorenza Maria Argolini: None declared, Davide Donzelli: None declared, Tamara Vojinovic: None declared, Véronique Ramoni: None declared, Elisa Bellis: None declared, Laura Trespidi: None declared, Federica Gazzola: None declared, Enrico Ferrazzi: None declared, Sonia Zatti: None declared, Fausta Benvenuti: None declared, Pier Luigi Meroni: None declared, Franco Franceschini: None declared, Carlomaurizio Montecucco: None declared, Rolando Cimaz: None declared, Roberto Caporali Consultant of: AbbVie; Gilead Sciences, Inc.; Lilly; Merck Sharp & Dohme; Celgene; Bristol-Myers Squibb; Pfizer; UCB, Speakers bureau: Abbvie; Bristol-Myers Squibb; Celgene; Lilly; Gilead Sciences, Inc; MSD; Pfizer; Roche; UCB, Angela Tincani: None declared

Background There are many differences in clinical manifestations, assessment and management of Juvenile Idiopathic Arthritis (JIA) between childhood and adults9 arthritis onset. The transition from pediatric to the adult care emphasizes a lot of aspects that need to be addressed. Objectives To describe the long-term outcome of JIA. Methods Two-hundred and forty patients affected by JIA and referred to a transition care rheumatology tertiary centre were considered between 1999 and 2016. The outcome assessment included disease activity, medications, number of prosthesis implantation, pregnancy, mortality, social integration (mobility, employment status and educational level). Results Seventy-four (30.8%) males and 166 (69.2%) females were included; 53 (22.1%) patients were lost in follow up. Subtypes of JIA at disease onset included 101 oligoarthirtis (42.1%), 67 polyarthritis (27.9%), 43 systemic arthritis (17.9%), 7 psoriatic arthritis (2.9%), 22 enthesitis related arthritis (9.2%). Forty-eight (20%) patients had persistent uveitis. Ninety-three implant prosthesis and 14 arthodesis were recordered. The average disease duration was 20 years, the median age of the patients was 27 (18–57) years. Five deaths (2.1%) occurred in this cohort. At follow up 117 (48.7%) had low active disease activity, 70 (29.2%) had moderate disease activity, 14 (5.8%) had a high disease activity, 24 (10%) were on remission ON medication and 15 (6.3%) OFF medication. Among patients still on medication, 59 (24.6%) were treated with oral steroids, 18 (7.5%) with csDMARDs and 169 (70.4%) with bDMARDs. Seventy-five (31.3%) patients had a higher educational level (university), 195 (81.3%) had an employment, 128 (53.3%) had a driving license. Twenty-one (8.8%) pregnancies were registered. The transition age was considered after age of sixteen years old. In this contest, it was important the multidisciplinary approach of each patient that was realized with the collaboration of other specialists (ophthalmologist, orthopedic, dermatologist, obstetric, psychologist). Conclusions In the era of biologic therapy there was an important improvement in a lot variables of the long-term outcome of JIA. One-hundred-eighty-seven (77.9%) patients were still in tight control, not only because of the continuation of the biological therapy but also because of the multidisciplinary care carried out even during remission. JIA often persists over the adulthood. The long term follow up and care of these patients has to be conducted by a rheumatologist expertized in JIA in collaboration with other specialists. Disclosure of Interest None declared

Background A potential role of hydroxychloroquine (HCQ) use in patients with antiphospholipid antibodies (aPL) has been suggested by some retrospective cohorts and in animal models based on its potential beneficial effects on autoantibody effect and titer, endothelial function and glucose or lipid metabolism [1–3]. Objectives To evaluate the impact of HCQ on aPL titers, on glucose and cholesterol levels and its role in the prevention of thrombotic recurrences in Primary Antiphospholipid Syndrome (PAPS). Methods Patients with PAPS, diagnosed according to 2006 Classification Criteria without any Connective Tissue Disease, that received HCQ (HCQ+) but no immunosuppressive drugs, were matched with similar patients that did not received HCQ (HCQ-) according to the following characteristics: same gender, follow-up duration, disease onset, age at the beginning of the follow-up ±10 years and initial date of the follow-up ± 5 years. Results Seventy-two patients (36 HCQ+ and 36 HCQ-) with a mean follow-up of 77 (±SD 49) months between 1992 and 2015 were studied. No significant variations in demographical, clinical, serological features were observed between the two groups except for of anti-extractable nuclear antigen antibodies (19% in HCQ+ versus 0% in HCQ-,p:0.014). Anti-β2Glycoprotein I IgG titers were significantly lower in HCQ+ than in HCQ- patients at the end of the follow-up (Student t,p:0.018). The variations of all the aPL titers in the two groups are shown in Figure 1. No differences in glucose and cholesterol levels were detected. Eight percent of HCQ+ and 17% of HCQ- patients experienced a thrombosis during the follow-up. Among patients with a history of thrombosis the annual incidence of recurrences was lower (0,87%) in HCQ+ patients than in HCQ- (2,17%). Conclusions This study showed a strong reduction of aPL titers and of the incidence of recurrences in patients treated with HCQ, supporting a possible role of this drug in the treatment of patients with PAPS. References Rand JH, Wu XX, Quinn AS, et al. Hydroxychloroquine directly reduces the binding of antiphospholipid antibody-beta2-glycoprotein I complexes to phospholipid bilayers. Blood 2008, 112:1687–1695. Broder A, Putterman C. Hydroxychloroquine use is associated with lower odds of persisently positive antiphospholipid antibodies and/or lupus anticoagulant in systemic lupus erythematosus. J Rheumatol 2013, 40:30–33. Schmidt-Tanguy A, Voswinkel J, Henrion D, et al. Antithrombotic effects of hydroxychloroquine in primary antiphospholipid syndrome patients. Journal of Thrombosis and Haemostasis. 2013;11(10):1927–1929. Disclosure of Interest None declared

Background During the last sixteen years, biologics have been used in our centre in an open prospective study for the treatment of refractory JIA not only in paediatric age but also in young adults. Objectives To underline the capability to become pregnant, not negatively influenced by biologics and to describe the pregnancy outcome of our young patients who got pregnant during the biologics treatment, since this event seems to be facilitated from a low disease activity under biologics. Methods Seventeen women affected by refractory JIA became pregnant during biologic therapy. Two males affected by JIA, both treated with Adalimumab have been achieved as indirect pregnancies. All patients had a long lasting refractory polyarticular disease not responsive to DMARDs. Patients were treated with biological agents like Etanercept (9 patients), Adalimumab (4 patients), Golimumab (3 patients), Infliximab (1 patient) and Rituximab (2 patients) as monotherapy and most of the cases after multiple switches. Results 3 patients decided for an elective termination; 16 patients had a normal pregnancy resulted in live born infants, full term, with no structural abnormalities and still in good health. The median time of exposure to biologics was 48 months (min 10- max 72 months). No abnormalities were achieved neither in the live born infants nor in the fetuses of the pregnancies which ended with an elective termination. The paternal exposure did not influence conception and was not associated with negative outcome of pregnancy. Conclusions The availability of therapies like antiTNFa and other biologics has raised important questions about pregnancy outcome in female and male patients affected by JIA exposed to biologics. Other data regarding limited populations affected by Crohn9s disease or Rheumatoid Arthritis treated with antiTNFa have been published. It doesn9t seem to be an increased risk to pregnancy loss. Even if there is no increase in miscarriage or malformation or birth defect, as reported in letterature, TNFa inhibitors are raccomended to be discontinued after pregnancy detection. In our experience no complications or unexpected side effects on the course of pregnancy were observed. Disclosure of Interest None declared