Soad Hashad

Objectives: Still disease, which includes systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still disease (AOSD), has historically had age-related treatment differences. With the advent of bDMARDs and evolving disease knowledge, management approaches have shifted. Our study aims to describe 2024 reported clinical practices for Still disease in France, and compare them with 2017 and 2024 national guidelines to identify practice gaps. Methods: This study is a French subanalysis of the European JIR-CliPS project, which evaluates reported prescribing practices for inflammatory diseases, taking into account recommendations and socio-economic factors in the participating countries. A detailed questionnaire was distributed to French physicians through professional societies and colleges. Results: Forty-seven physicians, mainly pediatric rheumatologists, internists, and rheumatologists, participated. While national guidelines influenced therapeutic decisions in most cases, only 26.7% followed them strictly, and 78.9% perceived no significant differences in the management of sJIA and AOSD. Treatment decisions were primarily guided by disease phenotype, severity, and relapse status. The use of bDMARDs as first-line treatment remained limited for several phenotypes, and glucocorticoid tapering strategies were heterogeneous, with most physicians adjusting tapering based on clinical response rather than following a standard schedule. Conclusion: This study highlights gaps between 2024 practice and current national guidelines for Still disease, particularly in first-line bDMARD use and glucocorticoid tapering. Understanding these gaps may support standardized practice and improved management of Still disease in the future.

Pediatric rheumatology is a relatively new specialty in the Arab world. It is still a growing field in which the number of centers and specialists is gradually increasing in these countries. Unfortunately, few published data are describing pediatric rheumatology in the Arab world. Therefore, we sought to provide an overview of pediatric rheumatology services in these countries, as well as to assess the published papers from Arab countries in this field. We gathered the information by conducting an online questionnaire on “SurveyMonkey.” We distributed the survey’s link via email and WhatsApp to the pediatric Arab rheumatology group. The responses provide an idea of the pediatric rheumatology practice. Data on academic pediatric rheumatology activities in the Arab world are scarce. In this chapter, we will highlight some of the remarkable publications that described the incidence and prevalence of juvenile idiopathic arthritis, which is one of the most common diseases in pediatric rheumatology in the Arab world, autoinflammatory conditions and some of the basic science publications. We will also focus on some challenges that pediatric rheumatology services face, such as a shortage of pediatric rheumatologists, limited access to diagnostic laboratory and medications, and the referral system. New emerging topics such as the COVID pandemic and telemedicine health in pediatric rheumatology will be discussed as well.

Rheumatic and musculoskeletal diseases (RMDs) represent a significant burden in Libya. Certain conditions, including systemic lupus erythematosus, Behçet disease, and familial Mediterranean fever, appear more prevalent than in Western countries, while polymyalgia rheumatica and spondyloarthritis appear less common. Epidemiological data remain scarce due to the absence of a national RMD registry. Rheumatology services began in the early 1990s and have since expanded to include adult and pediatric departments in Tripoli and Benghazi. The Libyan Rheumatology Society plays a central role in education and specialty development since its founding in 2002. Despite these advances, the ongoing political instability following the 2011 revolution has severely disrupted healthcare infrastructure, contributed to a significant brain drain of rheumatologists, and prevented the establishment of formal training programs, national screening initiatives, and accredited centers of excellence. Key genetic diagnostic tests remain unavailable locally. Access to biologic therapies is confined to a few major cities, requiring patients from other regions to travel for treatment. Political stabilization and targeted investment in rheumatology infrastructure, training, and research are essential to improving outcomes for RMD patients in Libya.

Objective To present updated evidence-based recommendations with a range of therapeutic options for children in Africa with oligoarticular juvenile idiopathic arthritis (JIA), adopting the treat to-target approach. Methods This study was conducted by the Paediatric Society of the African League Against Rheumatism (PAFLAR) to reach a consensus for recommendations regarding diagnosis and management of oligoarticular JIA suitable for application among different African countries. The Delphi technique was used to reach a consensus of 15 key questions identified by the steering committee. According to the PICO (patient/population, intervention, comparison, outcome) approach, a voting process among 22 African and international clinicians and experts in the field of pediatric rheumatology was performed after intensive systematic review of literature. Results A consensus was reached for 15 recommendations for diagnosis, monitoring, management of the disease, and its complications, intervention, and when to discontinue treatment of oligoarticular JIA patients with high level of agreement (ranging between 85 and 95%) who agreed or strongly agreed, and the response rate was 100%. Conclusion These recommendations focus on the early diagnosis and multidisciplinary management of oligoarticular JIA and its complications with high levels of consensus and agreement, improving practice consistency among healthcare providers. Key Points • A panel of experts created a consensus about the early diagnosis of oligoarticular JIA • Consensus-based recommendations to the management of oligoarticular JIA • These guidelines emphasized on the oligoarticular JIA-specific characteristics, severity factors, and treatment alternatives

The objective of this study is to develop evidence-based recommendations for the diagnosis and management of enthesitis-related arthritis (ERA) and juvenile psoriatic arthritis (JPsA) in the African context. The recommendations for ERA and JPsA were combined into a single document. The steering committee and task force identified 15 key questions and formulated 35 research questions. A comprehensive literature review, utilizing Medline and a manual search for African local data, was conducted to gather evidence. Following this synthesis, the task force developed draft recommendations and engaged in a Delphi process with an expert panel, including 17 African and three international experts, to reach a consensus and ensure alignment with global standards. The final recommendations were assigned a level of evidence and subsequently approved by the task force members, the expert panel, and the PAFLAR Board. Fifteen recommendations on the diagnosis and management of ERA and JPsA were developed, covering the role of the pediatric rheumatologist in multiple aspects of disease management, including diagnosis, monitoring of disease and extra-articular manifestations, determining treatment strategies, and guiding interventions. The level of evidence supporting these recommendations was variable, leading to the identification of a research agenda to address African particularities and answer pending questions. The final recommendations achieved a high level of agreement, with consensus ranging from 90 to 100%. These recommendations represent an important achievement for pediatric rheumatology in Africa, being the first of their kind, tailored specifically to the region. Developed through a rigorous methodology and collaboration between international and African experts, they aim to standardize care and address the unique challenges faced in African setting.

Hend Alkwai, M.D., Reem Alshammari, M.D., Reem Abdwani, M.D., Muna Almutairi, M.D., Raed Alzyoud, M.D., Thaschawee Arkachaisri, M.D., Sumaira Farman, M.D., Soad Hashad, M.D., Rebecca James, M.D., Khulood Khawaja, M.D., Hala Lotfy, M.D., Swee Ping Tang, M.D., Soamarat Vilaiyuk, M.D., Sulaiman M Al-Mayouf, M.D.. J Rheum Dis 2024;31:223-9. https://doi.org/10.4078/jrd.2023.0071

Juvenile psoriatic arthritis (JPsA) is a relatively rare condition in childhood as it represents ∼5% of the whole Juvenile Idiopathic Arthritis (JIA). There are fewer reports describing the characteristics and outcome of patients with JPsA. To describe the characteristic features and the treatment modalities of JPsA among Libyan children in pediatric rheumatology clinic at the Tripoli Children Hospital which is the only referral clinic covering the western, northern and southern part of Libya and to compare our results with other populations worldwide. All the medical records of the children who were diagnosed to have JPsA according to ILAR classification criteria or Vancouver criteria (definite or probable) from January 2001 to January 2020 were included and retrospectively reviewed. Data were collected about demographic, clinical, and laboratory features and treatment The study included a total of 30 cases of JPsA that represents a 12% of total JIA cases over the study period, the mean age at presentation was 5.8 ± 5.3 years with male to female ratio of 1:1, with the mean duration of symptoms prior to diagnosis of JIA of 9 ± 10 months. The foremost common type of arthritis was peripheral polyarthritis (83% & 58.3%), with the Small joints commonly affected, 30% of patients had hip joint involvement. 13% of the patient had psoriasis, half of them before presentation of arthritis. Nail changes secondary to psoriasis was seen in 1 of the cohort, and the percentage of patients with dactylitis among the group was 30%. Family history of psoriasis found in 83% of the cohort, 26% found to be ANA positive. Chronic uveitis occurred in 10%. Patients of the total cohort, ANA positive, Unilateral anterior uveitis being the most common presentation. The following table summarizes the Medications used during the course of the disease At the time of the first visit 100%of total patients fulfilled the Vancouver criteria for juvenile PsA, While 5 of the12 patients didn’t fulfilled the ILAR criteria for JPsA. Juvenile psoriatic arthritis constituted 12% of our patients with juvenile idiopathic arthritis. Juvenile psoriatic arthritis constitutes 8% to20% of JIA patients worldwide with equal gender distribution and peak age at presentation of 6–13 years. More than four peripheral joints involvement were the most frequent presentation. Half of the patients had skin involvement before the development of arthritis. Prevalence of chronic anterior uveitis was higher in our cohort than worldwide (10% vs 5% respectively) as an extra articular manifestation. All patients were treated with NSAIDs at presentation and/or during follow-up. Methotrexate was the most commonly used second-line agent Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

Systemic juvenile idiopathic arthritis (sJIA) constitutes a small part of juvenile idiopathic arthritis (JIA), yet has a disproportionally higher rate of mortality. In comparison to the other (JIA), systemic juvenile idiopathic arthritis has many distinct clinical features that arthritis may be a non-prominent and/or a delayed manifestation. The Pediatric Rheumatology International Trial Organization (PRINTO), currently is developing a new criterion for JIA classification which will allow for better identification of sJIA cases with delayed onset of arthritis and fail to fulfil the currently used International League Against Rheumatism (ILAR) and Yamaguchi criteria. It is useful in classification of children with systemic juvenile idiopathic arthritis (sJIA) particularly in ‘pre-arthritic’, pure systemic phase of the illness. The purpose of this study is to compare the performances of the Yamaguchi diagnostic criteria vs PRINTO criteria vs current ILAR criteria for systemic juvenile idiopathic arthritis. We compared the proportions of patients classified and how closely the 3 instruments are related. The study was performed at the Department of Paediatric Rheumatology in Tripoli children hospital. It is a retrospective study, covering the date range 2006–2020. We used clinicobiologic data recorded within 7 months of diagnosis of Patients diagnosed with sJIA. We assigned a diagnosis of sJIA according to the 3 sJIA classification systems (the Yamaguchi diagnostic criteria, PRINTO criteria and according to ILAR classification). Three different classification criteria were analysed in 38 assigned sJIA children (13% of total JIA cases). Females were 28 (52.6%), and mean age of JIA onset was 7 ± 3.9 yr. The mean of the time delay between disease onset and diagnosis was 7 ± 9 months. Overall, the ILAR and PRINTO criteria were fulfilled in a higher number of patients in the study (n = 35) (92% of patients), As compared with the Yamaguchi criteria (n = 29).(76% of patients). Among the 7.9% patients who were unclassifiable using the ILAR criteria were diagnosed as sJIA by both the PRINTO and the Yamaguchi diagnostic criteria. 7.89% neither fulfilled the Yamaguchi or the PRINTO Criteria. The table below compares the performance of the three different criteria. The performance of the 3 different criteria in the diagnosis of sJIA PRINTO: Pediatric Rheumatology International Trial Organization, ILAR: International League Against Rheumatism, PPV: positive predictive value, NPV: negative predictive value 7.9% subset of patients were in the ‘pre-arthritic’ phase of the disease at diagnosis and those patients were classified as sJIA by both the Yamaguchi criteria and the PRINTO criteria but not the ILAR criteria The performance of the 3 different criteria in the diagnosis of sJIA PRINTO: Pediatric Rheumatology International Trial Organization, ILAR: International League Against Rheumatism, PPV: positive predictive value, NPV: negative predictive value 7.9% subset of patients were in the ‘pre-arthritic’ phase of the disease at diagnosis and those patients were classified as sJIA by both the Yamaguchi criteria and the PRINTO criteria but not the ILAR criteria In this cohort the ILAR criteria appear to be superior in the early recognition and classification of sJIA. Yamaguchi diagnostic criteria can only be used to classify a minority of sJIA patients, leaving a large proportion of sJIA patients unclassified. Compared with the Yamaguchi criteria, ILAR JIA categories aligned better with PRINTO criteria. All the criteria should incorporate strengths of each other. Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

Macrophage Activation Syndrome (MAS) in Systemic Juvenile Idiopathic Arthritis (SoJIA) is a potentially life-threatening condition. The prevalence of MAS in SoJIA population is estimated to be 10%. However, reports suggested that the number of SoJIA patients with subclinical MAS may be as high as 40%. Early recognition of MAS is crucial because timely treatment is important for survival. To describe the clinical and laboratory features of patients with MAS associated with SoJIA Data from cohort of 38 SoJIA patients with and without MAS from January 2006 to January 2020 were included and retrospectively reviewed. Data were collected on demography, clinical/laboratory features and treatment. There were 38 patients with SoJIA diagnosed according to the International League Against Rheumatism (ILAR) criteria. 47.4% of them were males and 52.6% were females. They were followed up for a period of 6.3 ± 4.4 years. The mean period from appearance of symptoms to first follow up in rheumatology clinic was 5 ± 6 months. The disease course was monocyclic in 39.5%, polycyclic in 28.9% and persistent in 31.6% of the patients. Of the total cohort, 10% of the patients were classified as having MAS, half of them presented with MAS at diagnosis and the rest developed MAS during the disease course. Only One patient experienced recurrent MAS (3 times). Male to female ratio is 3:1. The mean age at diagnosis was 8 ± 6 years. Skin rash was the most presenting feature in the two groups. Hepatosplenomegaly and serositis were most common in sJIA with MAS patients. In comparison to SoJIA patients without MAS, all patients have thrombocytopenia, leukopenias, with mean ferritin level of 1668 in MAS patient and 947 in non-MAS group. The obligate criteria for the diagnosis of MAS according to the 2016 MAS classification criteria are increased ferritin >684 μg/l, elevated lactate dehydrogenase (LDH) and Glutamic Oxaloacetic Transaminase (GOT). All patients have a declining ESR and high CRP and all patients were anaemic. Bone marrow biopsy was done in 50% of the patients. In SoJIA without MAS, ESR mean level was 94 (±38.5) mm/h, CRP mean level of 62 (± 65.8) mg/dl and platelets count mean level of 561 (±215) × 10⁹/l. all MAS-SoJIA patients were treated with IV methylprednisolone and IVIG compared with 50% of non-MAS-SoJIA patients. In the MAS group, dose of oral prednisolone was (1–2 mg/kg/day) while in non-MAS group was (0.5–1 mg/kg/day). A patient developed MAS twice while on the 7th dose of anakinra and the third MAS on the 6th dose of tocilizumab. Ciclosporin (3 mg per/kg/day) was the treatment of choice for him. In our series, prevalence of MAS was 10% which mainly affect the male which is the same as the estimated prevalence of MAS worldwide. Arthritis occurred less frequent in MAS group cases in comparison to hepatosplenomegaly which was more common among MAS group. Rash and serositis were the most common extra-articular manifestations. The main treatment modality was steroid in all of the patients. All the MAS patients were successfully treated with high dose of IVIG, methylprednisolone and ciclosporin, including the patients who developed MAS while on biologics. Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

Introduction: Primary vasculitis syndromes are rare in childhood. The clinical manifestations depend on the vessels involved and can lead to significant mortality and morbidity. Aim: To study demographic and clinical characteristics of patients with diagnosed with vasculitis other than Kawazaki disease and Henoch-Schonlein purpora (HSP) and to report the outcome of the patients. Materials and methods: This is a case series conducted by reviewing the files of patients diagnosed with vasculitis and followed up at the pediatric rheumatology unit of the Tripoli Children Hospital, Libya from year 2000–2022. Data regarding patients’ demography, time of diagnosis, clinical features, laboratory results, treatment and complications were retrieved. Results: A total of 37 cases included in the study. 17 (45.9%) were males and 20 (54%) were females followed up for a mean period of 4.48 ± 4.21. Mean age at diagnosis was 8.39 ± 3.84 years. Regarding the diagnosis, there were 3 (8.1%) patients with Takayasu arteritis, 5 (13.5%) patients with polyarteritis nodosa, 2 (5.4%) patients with cutaneous polyarthritis nodosa, 1 (2.7%) patients with granulomatosis with polyahgitis, 1 (2.7%) patient with microscopic polyangiitis, 3 (8.1%) patients with Chrug-Strauss disease, 16 (43.2%) patients with Behcet disease, 1 (2.7%) patients with cogan syndrome, 3(8.1%) patients with urticarial vasculitis, 1 (2.7%) patient with hypocomplementaemic vasculitis, and 1 (2.7%) patient with DADA2. Mean period between the start of symptoms and diagnosis was 1.4 ± 2.8 years. In 6 (16.2%) of the patients there was family history of vasculitis and in 11 (29.7%) the parents are consanguineous. Angiography revealed abnormalities in 5 (13.5%) of the patients. Skin biopsy was done in 4 (10.8%) patients [2 patients with polyarthritis nodosa and 2 patients with urticarial vasculitis] and renal biopsy was done in 1 (2.7%) patient. Steroid was used in 24 (64.9%), Azathioprine in 4 (10.8%) patients. Cellcept was used in 10 (27%) of the patients. Ciclosporin was used 1(2.7%) patient, cyclophosphamide in 3 (8.1%) patients and Immunoglobulin in 6 (16.2%) of patients. Biologics were used in six patients, adalimumab in one patient which was discontinued because of recurrent abscess. In the two patients who used infliximab, treatment was effective. Three patients were treated with tocilizumab, it was ineffective in two, therefore discontinued. However, tocilizumab was effective in one patient with Takayasu arteritis. Complications developed in 23 (62.2%). Muscle atrophy in 1, deforming arthritis in 1, osteoporosis in 1, mouth ulcers in 2, hypertension in 2, hearing loss in 1, chronic sinusitis in 1, cardiovascular accident in 1, cataract in 1, visual impairment in 3, proteinuria in 2, pulmonary fibrosis in 1, chronic asthma in 3, impaired renal function in 1. Conclusion: This is the first report of pediatric vasculitis cases in Libya. It showed a heterogeneous spectrum of vasculitis with high rate of consanguinity and low rate of reported family history of vasculitis. Hopefully this report will increase awareness among clinicians of vasculitis and more cases will be diagnosed.