O002. Factors facilitating the self- and shared-management of Juvenile Idiopathic Arthritis

Background Juvenile idiopathic arthritis (JIA) requires some form of lifelong management, with at least one third of children symptomatic in adulthood. Therefore, empowering children to competently self-manage their health and wellbeing across the lifecourse is logical, while supporting families in their shared-management role during childhood. However, there was a limited theoretical basis to the self- and shared-management of JIA across the lifecourse. The objective is to explore the factors facilitating the self- and shared-management of JIA using a realist approach to evaluation. Methods Guided by the Individual and Family Self-management Theory, a three-stage realist approach to evaluation was undertaken: 1) initial JIA self- and shared-management question theories were elicited from literature reviews and stakeholder insights [1]; 2) seven initial question theories were tested using teacher-learner cycle interviews with 20 participants; 3) findings were analysed using a theory-driven approach to thematic analysis, using deductive, inductive, and retroductive reasoning to extend or refute the initial question theories, in order to identify demi-regularities in the data. Results Six refined JIA self- and shared-management question theories emerged: 1) meaningful and bespoke self-management support across the life course for children and young people with JIA; 2) recognised and valued shared-management support for the families of children and young people with JIA, with autonomy in mind; 3) individual healthcare plans as a shared management communication tool to facilitate optimal management of JIA; 4) consistent recognition, value, and encourage of self- and shared-management support from the paediatric rheumatology multi-disciplinary team and associated professionals; 5) child, young-person, and family-focused paediatric rheumatology care and support services across the lifecourse; and 6) bespoke and inclusive approaches by education providers to enable children and young people with JIA to feel safe, supported, and able to fulfil their potential. Conclusion There is an increasing recognition of the importance of self- and shared-management of JIA and other paediatric-onset chronic conditions. However, there is a lack of an overall, cohesive approach to self- and shared-management between healthcare providers, education providers, and patient/parent organisations. The findings from this study illuminate the factors facilitating JIA self- and shared-management at individual, interpersonal, institutional and infrastructural levels, bearing relevance to individuals and organisations involved in caring for, and supporting children with JIA and their families.

P046 A review and retrospective case series of paediatric Sjögren’s syndrome from Southern Africa

Background Paediatric Sjögren’s syndrome (pSS) is an uncommon autoimmune paediatric disease, rarely reported in children in Africa. It remains an important consideration in a child with parotid swelling. Here, we present a retrospective case-series of four patients from a paediatric rheumatology clinic in South Africa and discuss some of the difficulties of diagnosis in our context. Methods We performed a retrospective analysis of patients who attend the Red Cross War Memorial Hospital Paediatric Rheumatology clinic between 2010 and 2019. Result The four patients diagnosed with Sjögren’s all had varied presentations and disease courses. There were 3 females and 1 male and the ages at diagnosis ranged from 6 to 19 years old. The time to diagnosis was prolonged and ranged from 1 month to 10 years. Two patients with primary pSS presented with extra-articular manifestations of arthritis, abdominal pain and fatigue, followed by dry mouth, dry eyes, parotid swelling and and suggestive histopathology. The remaining 2 patients had a secondary pSS due to juvenile onset SLE and tuberculosis respectively. Conclusion In less resourced settings the diagnosis of pSS is often delayed. Both patients with primary pSS had preceding extra-glandular manifestations. In less resourced settings, infectious diseases may present as pSS and associated rheumatic diseases causing secondary pSS must be considered.

P090 Rheumatic symptoms as the first manifestations of underlying systemic disorders

Background Joint and limb pain is a common presenting complaint within the paediatric population, affecting 10–20% of school-age children. They are also a major reason for referral to paediatric rheumatology clinics (1). The differential diagnosis for joint pain and/or swelling can include both benign and malignant causes. Studies have found that < =1% of musculoskeletal complaints are caused by neoplasia, mostly acute lymphoblastic leukaemia (ALL) (1). It is essential to exclude life-threatening conditions such as infections and malignancies before initiation of treatment for presumptive Juvenile Idiopathic Arthritis (JIA) (2). The objective of this case series is to highlight the musculoskeletal features and the clinical course among children with haematological malignancies. Methods Charts of patients referred from rheumatology clinic to the haemato-oncology clinic were selected. A detailed chart review was then carried out to obtain information on clinical presentation, diagnostic work up and clinical progress of the patient. Results Case 1 CS, A 2 year and 2 months old girl who presented to paediatric rheumatology clinic with 5 month history of lower limb pain associated with a fever of 40’C. The pain progressively worsened leading to inability to walk. She had previously been treated for Idiopathic Thrombocytopenic Purpura with steroids. Initial Bone Marrow Aspiration (BMA) had ruled out leukaemia. Four months later, her condition had worsened and was referred to our facility where a peripheral blood film and flow cytometry confirmed B cell ALL. Treatment was initiated but unfortunately the patient’s condition deteriorated and eventually passed away. Case 2 NM, a 2 year and 10 months old girl presented to our paediatric rheumatology clinic with bilateral foot pain for 8 weeks. She was initially limping and progressed to inability to walk within a month. Rheumatology review in Tanzania diagnosed the patient as JIA and started on steroids. On examination, systemic findings were normal except bilateral ankle joint effusion and tenderness. An X-ray displayed bilateral transverse lucencies in the metaphyseal ends of the tibia and fibula. BMA was done and confirmed ALL. Treatment was initiated and the patient attained remission after 18 months of therapy. She remains well 2 years after completion of therapy. Case 3 NC, a 4-year-old girl, presented with multiple joint pains for 2 weeks. Pain was associated with night awakening and recurrent fevers. C-Reactive Protein (CRP) was persistently high and was treated with several courses of antibiotics. She was assessed by a haemato-oncologist with unremarkable findings. A decision was made to do a BMA and flow cytometry, which confirmed B cell ALL. Conclusion Musculoskeletal complaints are frequently the initial manifestations of acute leukaemia in childhood (60%) and should be considered in the differential diagnosis of JIA (3). Initially laboratory test results can be normal therefore it is necessary to follow these children closely and request serial tests, until there is a clearly defined diagnosis of neoplasia. Finally, malignancy should be eliminated before introduction of immunosuppressive therapy as the use of steroids may mask and delay its diagnosis (2).

P044 Have we been missing cases of Juvenile Systemic Lupus Erythematosus in Nigeria? The experience of a paediatric rheumatology trainee in Lagos

Background Juvenile Systemic lupus erythematosus (JSLE) is a chronic multisystem autoimmune disease of childhood, which accounts for 10% to20% of systemic lupus erythematosus (SLE). It was initially thought that systemic lupus erythematosus (SLE), including JSLE, was rare in Blacks, this was eventually debunked with increasing reports from Africa. However, it is now known that SLE is more common among patients of African descent in western countries. While the estimated prevalence of JSLE in the developed countries is 0.36–2.5 per 100 000, data in Black Africans is scarce due to missed diagnosis, poor diagnostic capacity and under-reporting. JSLE has protean manifestations similar to common paediatric conditions such as severe malaria, overwhelming septicaemia, hyper-haemolytic crisis in sickle cell anaemia etc., which often cause delayed or missed diagnosis. The objective is to describe the demographic and clinical characteristics, including outcome of children with JSLE, thus raising awareness on their occurrence and management in Nigerian children. Methods Retrospective review of records of children diagnosed with SLE at the Adult/paediatric Rheumatology Clinic and Paediatric Wards of Lagos State University Teaching Hospital (LASUTH) from May 2018 to May 2021. Results Twenty-two children, nineteen (n = 19) girls and three (n = 3) boys, aged 5–17 years, fulfilled the American College of Rheumatology (ACR)’s diagnostic criteria for JSLE out of 45 children newly diagnosed with paediatric rheumatic diseases during this period. The duration of symptoms before diagnosis ranged from two weeks to three years. The presentations included recurrent severe anaemia (n = 16), arthritis (n = 17), arthralgia (n = 17), malar rash (n = 17), neurologic symptoms (n = 5) oral ulcers (n = 17), cardiopulmonary symptoms (n = 5), photosensitivity (n = 10) and renal disease (n = 14). Laboratory findings included elevated ESR with a mean (±SD) of 99.68 ± 44.44, positive ANA (n = 22), positive anti-dsDNA (n = 12), low C3 & C4 (n = 2), positive anti-Smith antibody (n = 8) and massive proteinuria (n = 14). All patients were treated with steroids and disease modifying anti-rheumatic drugs (synthetics and biologics) based on disease severity and organ manifestations. Sepsis (n = 4) was the most common preliminary diagnosis before a final diagnosis of JSLE was made, other preliminary diagnosis were pulmonary tuberculosis (n = 1), dermatitis (n = 1), acute glomerulonephritis (n = 1), Typhoid fever (n = 1), malaria (n = 1), deep vein thrombosis (n = 1), seizure disorder (n = 1), leukaemia (n = 1), meningitis (n = 1), meningoencephalitis (n = 1), hyper-haemolytic crisis in sickle cell anaemia (n = 1), Steven Johnson syndrome (n = 1), Juvenile Idiopathic Arthritis (n = 2), Eczema (n = 1), unexplained anaemia (n = 1) and acute rheumatic fever (n = 1). One boy and three girls defaulted from clinic after commencement of treatment due to severe financial constraints of their parents and religious beliefs, however six girls died, four from an acute flare and two from end stage renal disease. Conclusion Our study has shown that JSLE has protean manifestations with a tendency to miss its diagnosis due to similarity of signs and symptoms with common childhood diseases in our environment. JSLE may not be as rare as commonly thought, thus its prompt diagnosis and treatment require a high index of clinical suspicion.

Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey

Childhood primary angiitis of the Central Nervous System (cPACNS) is a rare autoimmune and inflammatory disease. It can result in significant neuronal damage, neurodevelopmental delay and potentially death. Childhood PACNS is divided into subcategories: angiography-positive p-cPACNS that affects medium and large vessels, and angiography-negative small vessel sv-cPACNS. Due to its rarity, variable clinical representation, and the lack of a diagnostic criteria and therapeutic plans, diagnosis and treatment of cPACNS is challenging and approaches vary. This survey collected information on diagnostic and therapeutic approaches to sv-PACNS. It was shared with international clinician networks, including the German Society for Paediatric Rheumatology, the Paediatric Rheumatology European Society, the “Network Paediatric Stroke,” and members of the American College of Rheumatology/CARRA Paediatric Rheumatology list server. This project has shown consensus in numerous diagnostic and therapeutic treatment approaches, highlighting key areas which will be utilised to develop statements in the use of expert consensus meetings to standardise diagnostic and therapeutic approaches in this rare inflammatory disease.

Henoch-Schönlein Purpura Following the First Dose of COVID-19 Viral Vector Vaccine: A Case Report

A 76 year-old female came to our observation one week after the vaccination with ChAdOx1 nCoV-19 AZD1222 for the onset of purpuric rash on her gluteal and legs regions associated with coxalgia and episodes of macrohaematuria. Henoch-Schönlein purpura (HSP) was diagnosed on the basis of the revised criteria developed by the European League Against Rheumatism, the Paediatric Rheumatology International Trials Organization, and the Paediatric Rheumatology European Society (EULAR/PRINTO/PRES). HSP is a common IgA-mediated small vessel vasculitis, typical of childhood, that affects several systems and is characterized by a tetrad of dermatological, abdominal, joint, and renal manifestations. The Etiology of HSP is not completely understood, but it was observed following upper respiratory tract infections, medications, vaccinations, and malignancies. HSP has previously been reported following immunization with various vaccines, mostly within 12 weeks post, suggesting a possible correlation. To our knowledge, this is the first report of the possible association between COVID-19 ChAdOx1 nCoV-19 AZD1222 and the onset of HSP in a previously healthy woman. No similar cases were reported amongst 23.848 participants in the ChAdOx1 nCoV-19 AZD1222 trial.

Identifying the content and context of pain within paediatric rheumatology healthcare professional curricula in the UK: a summative content analysis

Background The curriculum for professionals working in paediatric rheumatology should include pain but it is unclear to what extent this currently occurs. The aim of this study was to identify pain-related curriculum content and the context in which pain is presented in educational and training documentation for healthcare professionals in this clinical speciality. Methods Core curricula documents from UK based professional organisations were identified in partnership with healthcare professionals. Documents were analysed using a summative content analysis approach. Key pain terms were quantified and weighted frequencies were used to explore narrative pain themes. Latent content was interpreted qualitatively to explore the context within which pain terms were positioned. Results Nine curriculum documents were identified and analysed from doctors, nurses, physiotherapists and occupational therapists specialising in paediatric rheumatology. Pain themes represented a mean percentage of 1.51% of text across all documents. Pain was rarely presented in the context of both inflammatory and non-inflammatory condition types despite being a common feature of each. Musculoskeletal pain was portrayed simply as a ‘somatic’ symptom, rather than as a complex phenomenon involving biological and psychosocial processes. Content around the assessment and management of pain was vague and inexplicit. Conclusion Current educational and training documentation in paediatric rheumatology do not include core pain topics. Curricula for these healthcare professionals would benefit from updates in contemporary pain theories and examples of in-context, evidence-based pain practices. This should be a priority starting point for optimising patient pain care in paediatric musculoskeletal healthcare.