Objectives To evaluate the impact of family history of autoimmune diseases (FHADs) on the clinical characteristics and outcome of juvenile idiopathic arthritis (JIA). Methods We retrospectively reviewed children with JIA seen in 7 pediatric rheumatology clinics from 6 Arab countries. All included patients met the International League of Associations for Rheumatology classification criteria for JIA and had a disease duration greater than 1 year. Data were collected at the last follow‐up visit and comprised clinical findings, including FHADs. Disease activity and disease damage were assessed by Juvenile Arthritis Multidimensional Assessment Report, and juvenile arthritis damage index (JADI) respectively. Disease activity was categorized as remission off treatment, remission on treatment, or active disease. Results A total of 349 (224 females) JIA patients with a disease duration of 5 (interquartile range 2.9‐7.5) years were included. The most frequent JIA categories were polyarticular JIA and oligoarticular JIA, followed by systemic JIA. There were 189 patients with FHADs and 160 patients without FHADs. The most frequent FHADs were diabetes mellitus (21.2%), JIA (18.5%), rheumatoid arthritis (12.7%). Among patients with FHADs, 140/189 (74.1%) achieved clinical remission, while 131/160 (81.9%) patients without FHDs had clinical remission (odds ratio [OR] = 1.2, 95% CI 0.97‐1.5). Rate of consanguinity, enthesitis‐related arthritis (ERA) and psoriatic arthritis were higher in patients with FHADs (OR = 0.6, 95% CI 0.4‐0.9 and OR = 1.2, 95% CI 1.1‐1.4). Also, articular JADI correlated significantly with presence of FHADs (OR = 1.1, 95% CI 1.0‐1.1). Conclusion This study shows that autoimmune diseases cluster within families of patients with JIA with a high proportion of ERA and psoriatic arthritis. JIA patients with FHADs are likely to have more disease damage.

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.

Background Juvenile Systemic Lupus Erythematous (JSLE) is a systemic autoimmune disorder with speckled manifestations that can emerge overstretched period of time and can affect any organ system, most frequently the skin, joints, kidneys, and the nervous, hematologic, and cardiovascular systems. The Aim is to examine the clinical features, serologic and laboratory characteristics associated with SLE. To probe and outline Clinical and Immunologic features of Incomplete Lupus Erythematous (ILE) patients who progressed from ILE to SLE. Methods The files of patients diagnosed as SLE in pediatric rheumatology clinic from 2001 to 5/2021 were retrospectively reviewed. Result Thirty SLE cases were included; Females were more prevalent with a female: male ratio of 14:1. Mean age at presentation 11 ± 4 years (range of 5 months-13 years), Disease onset was before sixth birthday in (7%), above twelve years in (40%) of the patients, and 53% of the patients was among 6 –12 years age group (31% & 37% of them fulfilled the SLICC & ACR criteria respectively at diagnosis) .The Mean duration between the onset of symptoms and SLE diagnosis was 6 months ±2 years. The Pre-pubertal age group presented early. At diagnosis, 50% of the patient got SLICC score criteria <4, on other hand 70% of the patients had ACR score criteria less than four Variable The most common presenting feature was arthritis (83%) (polyarticular arthritis) followed by dermatological manifestations (46%) photosensitivity, malar rash, and discoid rash in order of most frequent, fatigability (37%), renal manifestation (23%) most commonly as hematuria, one case diagnosed as lupus nephritis by renal biopsy. ANA was positive in 87%, anti-ds-DNA positive in 40% and Anti sm positive in 17%. The most frequently used medications were steroids and hydroxychlorqiune, the most commonly used steroid sparing medications were azathioprine (43%), Mycophenolate mofetil (40%) and ciclosporin (13%), 26% were on antihypertensive, 3% required rituximab to control their disease and one patient received Eltromboag to treat refractory thrombocytopenia. the mean follow-up duration 46.9 ± 43.6m, 20% lost follow up, 13% died due to disease complications (renal system involvement, thrombocytopenia and neurological system involvements). Conclusion jSLE in Libya is very rare before the sixth birthday and presented early among 6–12 years age group with a delay of less than one year between the first presentation and time of diagnosis. SLICC criteria was sufficient to diagnose the disease in > 50% of patients. High index of suspension should be maintained because in some patient’s years may be passed before fulfilling the diagnostic criteria. The most common cause of death is renal involvement. Glucocorticoids are the backbone of jSLE treatment in the acute phase. Both azathioprine and Mycophenolate mofetil are sufficient to control the disease in most patients. The disease outcome is accepted in our cohort as most patients have mild disease activity with low dose steroid and a steroid-sparing agent.