Publication only abstractsPU01. Tuberculosis after Tocilizumab in SJIAJ. N. Bathia, P. PalDepartment of Pediatric Rheumatology, Institute of Child Health, Kolkata, India Correspondence: J. N. BathiaIntroduction: Tuberculosis is a major cause of morbidity in low an middle income countries. Antitumour necrosis factor targeted agents (anti TNFs) are known to increase the risk of reactivation of latent tuberculosis infection (LTBI). However, the risk in non anti TNF biologics such as Interleukin 6 inhibitor Tocilizumab (TCZ) has been noted to be very low or absent. Sporadic cases have been reported. One may be question if it is primary TB infection or LTBI reactivation post TCZObjectives: We present a 11 years old girl who was diagnosed with systemic onset juvenile idiopathic arthritis and given TCZ. However, she developed fever after the first dose which was subsequently diagnosed as tuberculosis.Methods: This 11 years old girl initially presented with a history of intermittent for 3 months which managed conservatively by antibiotics and antipyretics. On examination we found that there was left sided cervical lymphadenopathy. Other examination was within normal limits. The patient was admitted for almost a month however the fever was still persistent. Investigations revealed anemia, progressively increasing leucocytosis with a neutrophilic preponderance. Urine and blood cultures were negative. Bone marrow examination and cervical lymph node biopsy was normal. tuberculosis was ruled out. USG of abdomen and CT scan of thorax were within normal limits. As there was a maternal history of Carcinoma a PET scan was done which was also normal. The child received multiple antibiotics however the fever persisted. She was discharged later on as the fever spikes decreased. Two months after that the fever spikes increased however this time there was an evanescent rash, cervical lymphadenopathy and and left knee diagnosis. Finally a diagnosis of Systemic onset juvenile idiopathic arthritis was made and the child started on subcutaneous methotrexate. Due to relapses leflunomide was added an subsequently TCZ administered. The fever reappeared along with cough.Results: A chest Xray was done which showed mediastinal widening. Contrast enhanced CT scan of thorax showed Nodular opacity in RLL with few small pulmonary nodules and conglomerated Mediastinal necrotic lymph nodes. The tuberculin sensitivity test was also positive. All this pointed towards pulmonary tuberculosis and anti tubercular therapy was started.Conclusion: Tocilizumab is a humanized anti-IL-6 receptor antibody which inhibits the binding of IL-6 to its receptors, IL 6 has both pro- and anti- inflammatory action and is involved in Th17 and Th22 cell differentiation which is critical for anti-mycobacterial activity. It is produced early during mycobacterial infection. It has been note that administration of tocilizumab is associated with a very low or absent risk of tuberculosis reactivation. In our case the child was initially screened for tuberculosis before the diagnosis of sJIA. However, after administration of TCZ she still developed tuberculosis. If this reactivation of latent tuberculosis or primary infection remains debatable.Disclosure of Interest: None declaredPU02. Chronic non bacterial osteomyelitis in 11 Indian children - a single center experienceD. B. Pandya, M. Aggarwal, S. SawhneyPediatric & Adolescent Rheumatology Department, Sir Gangaram Hospital , New Delhi , India Correspondence: D. B. Pandya Introduction: Chronic non-bacterial osteomyelitis (CNO) is an autoinflammatory bone disorder usually affecting children and adolescents. CNO covers a wide clinical spectrum from rather mild, time-limited, monofocal bone inflammation to severe chronically active or recurrent multifocal bone inflammation. Clinical signs of bone inflammation include localized skin redness (rare), warmth and/or swelling, and pain. CNO is a diagnosis of exclusion. MRI is a choice of investigation in diagnosis , follow up and exclusion of other close mimics. Management usually involves non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, disease-modifying anti-rheumatic drugs (DMARDs, usually methotrexate or sulfasalazine), anti-TNF agents or bisphosphonates and anti IL-1 agents especially in monogenic forms of CNO. Objectives: We aim to unveil characteristics of CNO in eleven Indian children at our center in India. Methods: This is a retrospective analysis of 11 children who visited our unit between Aug 2013 to March 2022 and were diagnosed with CNO after excluding close mimics. Our collected data includes demographics , clinical presentation , management and follow up details. Results: This Table Shows Characteristics of 11 Indian Children with CNO at our Unit Conclusion: Pre-adolescent girls are most commonly affected. Tibia is the most common bone involved and metaphysis is the most common site of involvement. Some of these patients presented with low grade fever and skin lesions. MRI and Bone Scan remains the first imaging modality. Most of the patients responded to adequate course of NSAIDs. Few patients required bisphosphonates and biologics to control the disease.Trial registration identifying number: 1. Curr Osteoporos Rep. 2017; 15(6): 542–554. Published online 2017 Oct 27. doi: 10.1007/s11914-017-0405-9Chronic Recurrent Multifocal Osteomyelitis : Presentation , Pathogenesis & TreatmentSigrun R. Hofmann,1 Franz Kapplusch,1 Hermann J. Girschick,2 Henner Morbach,3 Jessica Pablik,4 Polly J. Ferguson,5 and Christian M. Hedrich1,6,7Patient Consent: Yes, I received consentDisclosure of Interest: None declared Table 1 (abstract PU02). See text for descriptionFull size tablePU03. Clinical and laboratory presentation of Neonatal Lupus Erythematosus (NLE) - case reportD. S. Lazarevic1,2, J. Vucic3, Z. Milosevic-Anđelkovic3, H. Stamenkovic1,2, T. Stankovic1,2, J. Vojinovic1,2 1Pediatrics, Faculty of Medicine, University of Nis, Nis, Serbia, 2Pediatric Rheumatology and Immunology, 3Neonatology Department, Clinic of Pediatrics, Clinical Center Nis, Nis, Serbia Correspondence: D. S. LazarevicIntroduction: Neonatal lupus erythematosus (NLE) is a rare acquired autoimmune condition that is present at birth with diverse clinical presentation. It is transient disorder with transplacental passage of mother’s antibodies. Often is misdiagnosed as intrauterine infection or sepsisObjectives: Clinical and laboratory presentation of newborn with NLE.Methods: We report a child with NLE in mother without positive history on autoimmune diseases.Results: We present premature male newborn, small for gestation age with clinical symptoms starting just after birth with leucopenia and thrombocytopenia, but without elevated inflammatory markers. Antibiotics were administrated despite all bacterial cultures were sterile. In the first weeks of life severe thrombocytopenia required administration of immunoglobulins and platelets transfusion, while erythematous rash on face has appeared. A detailed hematological and the most common neonatal virological diagnostic testing were performed. AIDS and syphilis were excluded. Serological tests have confirmed active herpes simplex infection and parenteral acyclovir were started until polymerase chain reaction (PCR) results were negative. Suspected immunodeficiencies and neonatal alloimmune thrombocytopenia were ruled out, as immunoglobulin levels and flowcytometry were normal. Within the first month skin rash have changed and extended on whole face and neck. Skin lesions becomes much reddish, annular and clearly demarked. Clinical spectrum with hematological disorders were enough to suspect on NLE. Anti-Ro, Anti-La and ANA antibodies in child have confirmed our diagnosis, while mother has and Anti dsDNA antibodies associated. ECG Holter monitoring have excluded congenital heart block. Only supportive treatment was advised.Conclusion: Neonatal lupus erythematosus should be considered in newborns with annular skin lesions, hematological disorders and without congenital heart block even in clinically healthy mothers.Patient Consent: Yes, I received consentDisclosure of Interest: None declaredPU04. Juvenile systemic lupus erythematosus – experience of a tertiary hospitalM. Bastos Gomes1, A. Costa Azevedo1, A. L. Carvalho2, I. Cardoso3, F. Aguiar4,5, M. Rodrigues4,5, S. Ganhão4, I. Brito4,5 1Pediatric Department, Unidade Local de Saúde do Alto Minho, Viana do Castelo, 2Pediatric Department, Hospital de Braga, Braga, 3Pediatric Department, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, 4Pediatric and Young Adult Rheumatology Unit, Centro Hospitalar Universitário de São João, 5Faculdade de Medicina da Universidade do Porto, Porto, Portugal Correspondence: M. RodriguesIntroduction: Juvenile systemic lupus erythematosus (jSLE) is a systemic autoimmune disease, which can affect several organs and systems, diagnosed in pediatric age. Despite representing 15-20% of SLE cases, it is characterized by a more severe course.Objectives: Characterize the population of patients with jSLE followed at a tertiary university hospital.Methods: Retrospective study, including the patients with jSLE followed at the Pediatric and Young Adult Rheumatology Unit, diagnosed between 1987 and 2021. Demographic, clinical and laboratory data were collected. Disease activity was evaluated using Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and SLE Disease Activity Score (SLE-DAS) (2018), and the disease damage was evaluated using the Systemic Lupus International Collaborating Clinics (SLICC) / American College of Rheumatology (ACR) damage index.Results: A total of 44 patients were included, 93% female, with a median age at diagnosis of 15 years (IQR 12-16) and median follow-up of 8 years (IQR 5-13). There were 2 deaths (sudden death; both with pulmonary hypertension (HTP)). The clinical manifestations were: mucocutaneous (72%), hematological (70%), osteoarticular (59%), renal (41% - class IV: 63%, class V: 21%), constitutional symptoms (27%), vascular (22%), serositis (16%), gastrointestinal (14%) and pulmonary (2%) manifestations. All patients had positive anti-nuclear antibodies, 59% with high title (>1:1000). Ongoing treatment was: glucocorticoids (70%), hydroxychloroquine (78%), mycophenolate mofetil (41%) and azathioprine (20%). 13% received rituximab. SLEDAI-2K at diagnosis was significantly higher than at last patient assessment (10 vs 2, respectively, p<0.001). Regarding disease activity, 87% have SLEDAI≤4 and 42% SLEDAI=0; SLE-DAS ≤2.08 in 80%, SLE-DAS ]2.08-7.64] in 9% and SLE-DAS>7.64 in 11%. SLICC/ACR damage index was ≥ 1 in 13%.Conclusion: The most frequent manifestations in this cohort were mucocutaneous and hematological, which is in agreement with the literature. Major organs were commonly affected, including 41% with renal involvement, corroborating the greater morbidity associated with this age group. Deceased patients had risk factors: one received a delayed diagnosis and had shrinking lung syndrome and HTP, the other had an overlap with systemic sclerosis and had severe early-onset HTP. Most patients have low disease activity, which is possible in the face of early diagnosis and timely and effective immunomodulatory and/or immunosuppressive therapy, as well as regular follow-up in a specialized unit.Patient Consent: Yes, I received consentDisclosure of Interest: None declaredPU05. The rare cause of back pain – the crucial role of ultrasound as the first step to diagnose Takayasu arteritisZ. Pytelová1, J. Čivrný2, T. Klimas2, D. Klepárník1, K. Bouchalová1 1Paediatric Rheumatology, Department of Paediatrics, 2Department of Imaging, Faculty of Medicine and Dentistry, Palacký University Olomouc and Olomouc Hospital, Olomouc, Czech Republic Correspondence: Z. PytelováIntroduction: Takayasu arteritis is a rare large-vessel vasculitis affecting the aorta, its major branches and the pulmonary artery, and common symptoms are non-specific myalgia, weight loss and fever or absence of peripheral pulse and claudication in later stages.Objectives: We aim to describe a case of a rare disease with early diagnosis and highlight the importance of thorough ultrasound imaging.Methods: We present a case report.Results: A 13-years-old girl was admitted for back pain lasting 1.5 months. The pain woke the girl up from sleep and there was no effect of NSAIDs. For a psoriatic lesion on her elbow, she was examined by a dermatologist once.On the day of admission, there were elevated inflammatory markers (CRP 172 mg/l, erythrocyte sedimentation rate, ESR 137 mm per hour), normocytic anemia and coagulopathy. During ultrasound imaging of the abdomen, the radiologist described signs of arteritis of the abdominal aorta. Immediately, CT angiography was performed with findings of involvement of abdominal aorta appropriate for inflammation process. The finding was assessed as Takayasu arteritis type 3 according to angiographic classification. MR angiography proved the diagnosis and will be used during follow-up. The patient received 5 pulses of corticosteroid therapy (methylprednisolone) followed by oral corticosteroid (prednisone). Infectious causes of inflammation were excluded. We observed a prompt improvement in clinical features.On the 12th day, we started treatment with tocilizumab 162 mg administered subcutaneously once a week.After 4 weeks, ultrasound finding was normal and we observed a decrease in inflammatory markers.At the time of abstract submission, she is symptoms-free, on therapy with tocilizumab, and her CRP and ESR are normal.Conclusion: Takayasu arteritis is rare large-vessel vasculitis. Common symptoms are non-specific and thus, there could be a delay in diagnosis. In this case, our radiologist noticed inflammatory changes in the abdominal aorta during the standard abdominal ultrasound and it led to a diagnosis. This case highlights both the importance of doing thorough ultrasound imaging and the effect of therapy – high-dose corticosteroid followed by biologics led to clinical improvement.Patient Consent: Yes, I received consentDisclosure of Interest: None declared