Hakan Kisaoglu

Objective: Juvenile idiopathic arthritis (JIA) is the most common chronic childhood arthritis, with distinct categories based on pathophysiology and clinical manifestations. The age of onset varies by category but is reported to be approximately six years. This study aimed to evaluate the clinical, laboratory and outcome characteristics of patients with infantile-onset JIA. Methods: This multicentre retrospective study included patients with JIA whose symptoms were onset before the age of two. Results: This study included 199 patients with a median age at symptom onset of 17 months and a median age at diagnosis of 20 months. The most common JIA categories were persistent oligoarticular (59.8%), systemic (13.6%) and rheumatoid factor (RF)-negative polyarticular (11.6%). Patients with symptom onset ≤12 months had a significantly higher prevalence of RF-negative polyarticular and systemic JIA, whereas those with onset >12 months had a higher prevalence of persistent oligoarticular JIA. Antinuclear antibody positivity was significantly higher in patients with disease onset >12 months, whereas uveitis development was more common in those with onset ≤12 months. The wrist, hand proximal interphalangeal (PIP) and foot PIP joints were significantly more frequently affected in patients with early-onset disease. At the last visit, 59.8% of the patients were in remission with medication and 18.1% were in remission without medication. Conclusion: This study suggests that patients with infantile-onset JIA exhibit distinct characteristics regarding JIA category, joint involvement and uveitis development, highlighting the need for further research and potential reconsideration of current age thresholds in the JIA classification.

Objective: This nationwide, multicenter study was conducted to assess the demographic and clinical features, treatment regimens, and prognosis of primary Sjögren disease (SjD) in childhood. Methods: This retrospective study included a total of 81 patients < 18 years of age from 21 pediatric rheumatology centers. Among these, 51 patients fulfilled the diagnosis of childhood SjD according to the 2016 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria. The remaining 30 patients—who did not fully meet these criteria but exhibited clinical and laboratory findings suggestive of SjD—were categorized as at risk for childhood SjD to highlight diagnostic challenges and the spectrum of early presentations, based on comprehensive clinical evaluation by experienced pediatric rheumatologists. Results: The cohort consisted of 81 patients (85.2% female, 14.8% male) with a median age at symptom onset of 11.4 years and a median follow-up of 24 months. Common clinical manifestations included dry mouth, dry eyes, arthralgia, fatigue, and parotitis. Fifty-one of the 81 patients met the 2016 ACR/EULAR classification criteria, whereas the remaining 30 were classified as an at-risk group. The most common clinical findings in the at-risk group were xerostomia (90%), arthralgia (56.7%), fatigue (50%), and dry eyes (43.3%). Dry mouth and peripheral nervous system involvements were found to be higher in patients categorized as at risk ( P = 0.03 and P = 0.02, respectively). Conclusion: The current classification criteria for childhood SjD appear to be inadequate, highlighting the need for pediatric-specific criteria that more accurately reflect the distinct clinical patterns observed in children.

Objective: To identify the feasibility of achieving the childhood definition of remission, investigate factors affecting achievement and determine the concordance rate with adult definition in children with systemic lupus erythematosus (SLE). Materials and Methods: Medical records of children diagnosed with SLE between 2012 and 2022 were reviewed. The Definitions of Remission in Systemic Lupus Erythematosus (DORIS) definition of remission was used as the adult definition of remission, and a lower glucocorticoid threshold, as proposed, was used for children weighing <50 kg. Cox regression analysis was performed to identify features associated with remission. Results: Among the 50 included patients, 35 (70%) achieved the adult definition of remission in a median of 16 months. While 33 (66%) patients achieved the childhood definition of remission, 25 (76%) achieved both definitions concomitantly. A lower rate of damage (15.2% vs. 52.9%, P= .008) and flare count (median 1 vs. 2, P=.001) were observed in patients with remission despite significantly longer follow-up duration (median 59 months vs. 32 months, P=.007). Survival analysis revealed that the presence of positive anti-dsDNA antibodies (hazard ratio [HR], 0.47; P=.035) and immunosuppressive usage (HR: 0.45, P =.032) were associated with a higher risk of not achieving remission. Conclusion: Childhood definition of remission is achievable in two-thirds of children with SLE and displays substantial concordance with the adult definition. Additionally, the higher risk of failure to achieve remission in children using immunosuppressants reflects a milder course in a subgroup of children who achieved remission and signifies the need for more efficacious treatment modalities for severe manifestations. Cite this article as: Kisaoglu H, Baba O, Kalyoncu M. Achievement and features associated with childhood definition of remission in juvenile-onset systemic lupus erythematosus. Turk Arch Pediatr. 2025;60(4):398-403.

Objectives: Management of systemic lupus erythematosus (SLE) through new treatment options has improved lupus nephritis (LN) prognosis. The aim of this study was to compare the changes in the demographic, laboratory, and treatment characteristics, prognosis, and outcomes of paediatric-onset LN patients over 30 years. Methods: We retrospectively reviewed the medical records of 103 paediatric-onset LN patients. Patients were divided into two subgroups according to the years of LN diagnosis. Group 1 consisted of patients diagnosed with LN between the years of 1993 and 2005, and group 2 consisted of patients diagnosed with LN between the years of 2006 and 2023. Results: The mean age at diagnosis of SLE, age at diagnosis of LN, time to LN development, and mean delay time to diagnosis were significantly higher in group 1 (P < 0.001, P < 0.001, P = 0.049, and P = 0.004, respectively). Baseline Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores were higher and anti-phospholipid antibody positivity was more frequent in group 1 (P = 0.040 and P = 0.025, respectively). Azathioprine in the maintenance phase was given more frequently in group 1 (P = 0.016), while rituximab was more frequently used in group 2 (P = 0.042). In both groups, the majority of the patients had proliferative LN (class III and/or class IV) (53.5% in group 1 vs. 68% in group 2). Complete renal remission was significantly more common in group 2 (P = 0.005), while end-stage kidney disease (ESKD) and death were significantly more common in group 1 (P = 0.005 and P = 0.001, respectively). Proteinuria and SLEDAI scores at the first visit were independent risk factors for progression to ESKD (P = 0.037 and P = 0.024). Conclusion: Over the years, there have been significant improvements in the diagnosis and management of children with SLE resulting in an earlier diagnosis, lower disease activity at onset, and improved outcomes.

Objective: To determine the feasibility and risk of flares by achieving successful glucocorticoid (GC) tapering during the first year of juvenile SLE and the value of early achievement of childhood lupus low disease activity state (cLLDAS). Methods: The medical charts of children with moderate-to-severe SLE between 2012 and 2022 were retrospectively analysed. Successful tapering was defined as the employment of a prednisolone equivalent dose, lower dose of either ≤7.5 mg/day or ≤0.15 mg/kg/day, as per the cLLDAS definition. A linear mixed-effects model was used to determine the fixed effects affecting the GC dose over the first year. Cox regression analysis was used to identify whether successful tapering increased the risk of flares, and logistic regression was used to determine the odds of flares after the twelfth month of treatment. Results: Successful GC tapering was observed in 50 out of 80 patients (62.5%) within the first year of treatment, and flares were observed in 23 (28.8%) patients. The GC tapering trajectories over time were similar based on flare observations (p>0.05). Furthermore, successful tapering did not increase the risk of flares. Additionally, patients without flares received significantly higher GC doses as the initial treatment (p=0.046). Achievement of cLLDAS was observed in 40 (50%) patients at the twelfth month; however, achievement was not protective against future flares, and positive anti-double-stranded DNA antibodies at the twelfth month increased the odds of flares (OR: 4.8, p=0.008). Conclusion: Successful GC tapering is feasible and does not increase the risk of flares during the early disease phase. However, flares are common and adversely affect GC tapering. Thus, the identification of children with an increased risk of flares on GC tapering is needed to reduce the GC burden.

Objective: Systemic lupus erythematosus (SLE) constitutes an autoimmune disorder with potential involvement of the gastrointestinal system (GIS). Our objective was to assess the gastrointestinal (GI) manifestations in patients diagnosed with childhood onset SLE. Methods: The study cohort consisted of 123 patients with childhood onset-SLE and GIS involvement from 16 referral departments of pediatric rheumatology. All participants met the Systemic Lupus International Collaborating Clinics criteria. Results: Out of 123 patients, 78 (63.4%) exhibited GIS involvement at the initial SLE diagnosis, whereas the remaining 45 (36.6%) developed GI symptoms after a median duration of 12 (3–140) months. Eighty-two (66.7%) individuals experienced symptoms related to the GI tract, whereas the remaining patients received a diagnosis of GI involvement through laboratory assessments. The predominant initial GIS involvement symptom was abdominal pain, observed in 77 (62.6%) patients, followed by elevated hepatic transaminases in 70 (56.9%), hepatomegaly in 40 (32.5%), diarrhea in 26 (21.1%), and jaundice in 11 (8.9%) patients. The GIS involvement was associated with SLE in 82 (78.6%), while it resulted from drug-related adverse events in 35 (28.5%) patients or comorbidities in 6 (0.5%) patients. Conclusion: GIS involvement should be considered in all childhood onset-SLE patients, especially in the presence of suggestive symptoms or elevated hepatic transaminases. It is also crucial to consider SLE in the differential diagnosis of GIS manifestations in children. Apart from GIS involvement directly associated with SLE, adverse events of drugs should be kept in mind.

Objectives: To investigate the severe haematological involvement in children with SLE and assess its clinical associations, treatments, outcome and damage accrual. Methods: The medical charts of children with SLE in whom haematological involvement was observed were reviewed. Severe haematological indices were defined as autoimmune haemolytic anaemia with a haemoglobin concentration < 8 g/dL, thrombocyte count < 30 000/µL, and neutrophil count < 500/µL. Results: Among the 224 patients included, 102 (45.5%) displayed severe indices, predominantly at the initial involvement, and most frequently as severe anaemia in 54 (24.1%) and severe thrombocytopenia in 45 (20.1%). Disease activity did not differ according to the presence of severe disease indices. In addition, the presence of severe indices at initial involvement did not affect the damage accrual. However, a higher rate of damage (51.1% vs. 29.9%, p = 0.002) and steroid-induced damage (28.9% vs. 8.2%, p < 0.001) was evident in patients with flares of the haematological system. Regression analysis revealed that rituximab treatment during the initial episode (OR:4.5, p = 0.006) and the presence of anticardiolipin antibodies (OR:2.3, p = 0.014) significantly increases the odds for haematological system flare. However, severe indices at initial involvement did not increase the odds of a haematological flare. Conclusion: Severe haematological indices at onset are common but not related with disease outcomes. Prevention of flares is important to improve outcomes, and a more rigorous maintenance strategy would benefit most to children who display haematological indices refractory to conventional immunosuppressants and those with anti-cardiolipin antibodies.