Elif Kilic Konte

Objective: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome of childhood, and growing evidence links early-life inflammation to neurodevelopment, but whether PFAPA affects attention and behavior at school age is unknown. This study compared parent-reported attention-deficit/hyperactivity disorder (ADHD) symptoms in school-aged children with a preschool PFAPA diagnosis with those of healthy peers and examined associations with PFAPA-related clinical characteristics. Materials and Methods: This cross-sectional study included 96 children (mean age 10.84 Å} 1.64 years) diagnosed with PFAPA during the preschool years and followed at a pediatric rheumatology clinic and 81 healthy controls (10.40 Å} 1.88 years) without chronic disease or tonsillectomy. Attention-deficit/hyperactivity disorder symptoms were assessed with the Revised Conners’ Parent Rating Scale–Short Form (CPRS-R:S). Results: The groups did not differ in age or sex. No significant between-group difference was found on any CPRS-R:S subscale. Within the PFAPA group, an earlier age at symptom onset was weakly but significantly correlated with a higher ADHD Index (ρ = −0.317, P = .002), and this association remained significant after false discovery rate correction (false discovery rate–adjusted P value [pFDR] = 0.048) and multivariable adjustment (β = −0.295, P = .004); subgroup analyses by comorbidity, tonsillectomy, and medication use showed no differences. Conclusion: Children with a preschool diagnosis of PFAPA did not show increased parentreported ADHD symptoms at school age. The weak association with earlier onset warrants cautious interpretation. These findings suggest that the immune–neurodevelopment relationship should be considered in a disease-specific manner, distinguishing PFAPA’s benign, episodic course from chronic autoinflammatory diseases.

To compare the sensitivity and prognostic value of SAA and C-reactive protein (CRP) during familial Mediterranean fever (FMF) attacks and attack-free periods. Patients with juvenile idiopathic arthritis (JIA), acute appendicitis, and sepsis were used as controls. In this prospective study, 42 patients with FMF, 30 with JIA, and 30 with acute appendicitis were included. Blood samples for CRP and SAA were collected at 2 different time points: first, during an attack or preoperatively for acute appendicitis cases, and second, after the subsidence of the attack or postoperatively. To evaluate the role of SAA and CRP in the follow-up of inflammation, 42 patients with sepsis were studied serially. Twenty healthy individuals were also tested as controls. In appendectomy, JIA, and FMF patients, the mean CRP and SAA levels returned to normal values after operation/attack treatment. In both the inflammatory and non-inflammatory phases, a positive correlation was detected between CRP and SAA levels ( P < .001). In the sepsis group, mean SAA and CRP levels were 221.21 ± 95,05 and 402.2 ± 255.4 respectively. CRP and SAA levels were consecutively measured in 19 patients with sepsis, and a positive correlation was detected between these markers ( P < 0.01). In healthy controls, the SAA and CRP levels were within the normal ranges. We report that SAA and CRP levels were correlated in various disease settings. We suggest that CRP measurements are adequate for evaluating acute inflammatory conditions, and follow-up with SAA does not contribute to the early recognition of subclinical inflammation.

Objectives: To evaluate age-related differences in clinical features, serological profiles, diagnostic approaches, treatment strategies and outcomes among juvenile- and adult-onset dermatomyositis (JDM and AoDM) patients managed at the same tertiary referral centre. Methods: This retrospective cohort included 180 patients with dermatomyositis meeting the 2017 EULAR/ACR classification criteria and followed between 1995 and 2025 at the paediatric and adult rheumatology clinics. Diagnosis before 16 years was classified as JDM. Results: Among 180 patients, 86 had JDM and 94 had AoDM. A bimodal age distribution was observed, with peaks at 6.8 and 45.7 years. 67.8% were female; median follow-up was 5.9 years. Muscle weakness (86.7%) and cutaneous involvement (98.3%) were most common findings. Calcinosis was frequent in JDM (33%) but rare in adults (one case). Both calcinosis and arthritis were significantly associated with JDM (P = 0.007 and P = 0.049). Anti-NXP2 was the leading JDM autoantibody (31.4%, 11/35), whereas anti-synthetase antibodies (34.6%, 9/26) in AoDM. Glucocorticoids were given to both groups; however, steroid-free (40.3% vs 89.6%, P = 0.020) and drug-free remission rates (40.3% vs 16.7%, P = 0.020) were higher in JDM. Malignancy-associated dermatomyositis occurred only in AoDM (n = 25, 26.6%). Age at diagnosis was independently associated with both mortality (OR 1.07 per year, 95% CI: 1.02–1.11) and malignancy (OR 1.05 per year, 95% CI: 1.00–1.10). Conclusion: JDM and AoDM are not merely age-defined variants but display distinct clinical, serological and prognostic profiles. JDM shows calcinosis and better remission and survival, whereas AoDM is strongly associated with malignancy and higher mortality burden, highlighting the need for age‑tailored diagnostic and management strategies.

Objectives: Canakinumab is widely used in colchicine-resistant familial Mediterranean fever (FMF), yet data on interval adjustment strategies and predictors of successful extension remain limited. This study evaluated clinical and inflammatory outcomes across dosing interval groups and identified factors associated with successful interval extension. Methods: This retrospective single-centre study included 60 pediatric patients with colchicine-resistant FMF treated with canakinumab. Patients were stratified into stable interval (group 1, n = 14), successful interval extension (group 2, n = 24) and unsuccessful interval extension (group 3, n = 22). Longitudinal C-reactive protein (CRP) trajectories were analysed using linear mixed-effects models. Clinical outcomes were compared across groups, and predictors of successful interval extension were assessed using multivariable logistic regression with Firth’s penalized method as a sensitivity analysis. Results: Following canakinumab initiation, CRP levels declined rapidly and stabilized after month 3, while the median annual attack rate decreased from 7 (4–12) to 1.1 (0.5–2.1) attacks per patient-year. Inflammatory trajectories did not differ across groups. Despite similar CRP dynamics, complete remission rates differed significantly (57.1%, 45.8% and 27.3% in Groups 1–3, respectively; P = 0.005), and all patients in group 3 experienced attacks. Arthritis at disease onset was independently associated with successful interval extension (OR = 7.14, 95% CI 1.26–40.41, P = 0.026), with consistent direction in Firth analysis (OR = 5.51, 95% CI 1.08–28.23, P = 0.081). Conclusion: Although canakinumab achieves sustained inflammatory suppression, biochemical improvement does not ensure comparable clinical control. Clinical phenotype, particularly arthritis at onset, may guide individualized interval adjustment.

Juvenile spondyloarthritis (JSpA) refers to a heterogeneous group of inflammatory diseases affecting peripheral joints and the axial skeleton during childhood and adolescence. Enthesitis-related arthritis (ERA) and juvenile psoriatic arthritis (JPsA), subtypes of juvenile idiopathic arthritis (JIA) under the International League of Associations for Rheumatology (ILAR) classification, are included in this group. They may be associated with human leukocyte antigen (HLA) B27 positivity. Apart from musculoskeletal involvement, pathologies that accompany the disease, such as psoriasis, uveitis, and inflammatory bowel disease, significantly affect patients’ quality of life and clinicians’ treatment approaches. Guidelines developed for the treatment of JSpA patients primarily address the use of nonsteroidal antiinflammatory drugs (NSAIDs), conventional disease-modifying antirheumatic drugs (csDMARDs), and tumour necrosis factor inhibitors (TNFi). However, not all patients respond adequately to or tolerate the currently available therapies, highlighting the need for novel treatment options. Recent advances in understanding immunopathogenesis have driven the development of new therapeutic targets, including TNF inhibitors, interleukin (IL)-17/IL-23 blockers, and Janus kinase inhibitors. Several of these agents, including secukinumab, have now received approval for the treatment of ERA and JPsA. However, since only a limited number of open-label and randomized controlled trials have specifically evaluated JSpA, current treatment strategies are also informed by evidence from other JIA subtypes and adult studies. Expanding the approved indications of existing biologics—supported by robust trial data—alongside the development of novel therapeutic agents, may broaden treatment options and ultimately improve outcomes for patients with JSpA.

Objective: The coexistence of psoriasis and juvenile‑onset systemic lupus erythematosus (jSLE) is rare, and data on familial aggregation of psoriasis across pediatric rheumatic diseases are limited. This study evaluated the co-existence of psoriasis in jSLE, its familial clustering, and its association with the jSLE phenotype. Methods: In this single‑center cross‑sectional study, patients with jSLE, familial Mediterranean fever (FMF), and juvenile systemic sclerosis (jSSc) were systematically screened for personal and family histories of psoriasis. Recurrence risk ratios for psoriasis were calculated separately for patients and their relatives within each disease group. Among patients with jSLE, clinical manifestations, laboratory parameters, and disease activity (SLEDAI‑2K) were compared between those with and without personal and/or familial psoriasis. Results: Of 189 patients with pediatric rheumatic diseases included, 94 (49.7%) had jSLE, 73 (38.6%) had FMF, and 22 (11.7%) had jSSc; 69.3% were female, with a higher frequency in jSLE patients (85.1%). Overall, 7,034 individuals (patients and relatives) were systematically screened for psoriasis. Psoriasis was identified in 4 patients (2.1%), all with jSLE (4.3%), and in 25 relatives (0.36%), yielding an overall prevalence of 0.41%. The combined prevalence of psoriasis in jSLE patients and their relatives (0.54%) was significantly higher than in FMF (0.35%) and jSSc (0%) families (p = 0.034). Recurrence risk ratios (λ) for psoriasis in jSLE families were 10.13 for patients and 3.07, 1.16, and 0.67 for first‑, second‑, and third‑degree relatives, respectively; corresponding λ values in FMF families were 0, 1.88, 1.11, and 0.45. Among 94 patients with jSLE, 20 had a personal or family history of psoriasis. However, their clinical signs, laboratory results, and SLEDAI-2K scores showed no significant differences compared with those without a history of psoriasis (p > 0.05). Conclusion: A positive family history of psoriasis is more common in jSLE than in FMF or jSSc, supporting the hypothesis of a shared genetic background between psoriasis and jSLE, but it was not associated with more severe jSLE in this cohort. These findings underscore the importance of routinely assessing familial psoriasis in jSLE and related disorders and warrant confirmation in larger, prospective, population‑based studies.

Tuberculosis (TB) remains a major global health concern, especially for children with rheumatic diseases on biologic therapies. While these agents have improved disease management and quality of life, they also pose new challenges, mainly an increased risk of TB, particularly with tumor necrosis factor‑alpha (TNF‑α) inhibitors like infliximab, adalimumab, and etanercept. Since TNF‑α plays a crucial role in granuloma formation and controlling Mycobacterium TB, its inhibition can allow latent TB infection (LTBI) to develop into active disease. To reduce this risk, screening for LTBI before starting biologic therapy is highly recommended. However, accurately detecting LTBI in children—especially those who are immunosuppressed—remains challenging, as both the tuberculin skin test (TST) and interferon‑gamma release assays (IGRAs) have notable limitations in this context. Using both TST and IGRA together may improve case detection in selected pediatric populations. Preventive treatment for LTBI reduces the risk of developing active TB in patients receiving biologic therapies. Although non-anti-TNF therapies may offer a safer option in this regard, the evidence in pediatric patients is still limited. As the use of biologic and targeted therapies expands in children, further research is needed—especially on non-anti-TNF agents—to create internationally accepted guidelines and to optimize screening, diagnostics, and personalized prevention strategies.

Idiopathic recurrent pericarditis (IRP) is an increasingly recognized inflammatory condition in children, characterized by recurrent flares and therapeutic challenges, with emerging evidence supporting a role for interleukin-1 (IL-1)-mediated pathways. This study aimed to characterize the clinical features of a pediatric IRP cohort at our center, to examine clinically relevant differences between patients requiring and not requiring anakinra, and to evaluate anakinra effectiveness by assessing changes in disease activity before and after treatment. In this single-center retrospective cohort study, 26 children with IRP were evaluated in terms of demographic, clinical, laboratory, genetic, and treatment characteristics. Patients were stratified according to anakinra requirement, and these characteristics were compared between groups. In patients receiving anakinra, treatment effectiveness was assessed by comparing disease activity before and after therapy using annualized attack rate (AAR) and inflammatory markers. The cohort showed a male predominance (19/26, 73%), with a median age at diagnosis of 114 months (IQR, 48–168). Chest pain was the most common presenting symptom (16/26, 62%). Pericardial effusion was present in all patients (26/26, 100%), while pleural effusion was observed in 12/26 (46%). Despite conventional therapy with nonsteroidal anti-inflammatory drugs (NSAIDs), colchicine, and corticosteroids, steroid dependency developed in 9/22 patients (41%). Anakinra was required in 16 patients (62%) and was associated with a marked reduction in disease activity, with a median relative decrease in AAR of 89.4% (IQR, 56.7–100%); a ≥ 50% reduction in AAR was achieved in 12/16 patients (75%). Significant and concordant reductions in inflammatory markers were observed. Relapse frequently occurred during anakinra dose spacing or discontinuation, while corticosteroid withdrawal was achieved in most treated patients. Patients requiring anakinra had higher baseline disease activity. Conclusion : Idiopathic recurrent pericarditis in children represents a heterogeneous and frequently relapsing inflammatory condition in which conventional therapies often fail to achieve sustained disease control. As an IL-1 blockade, anakinra emerges as a key therapeutic option, enabling corticosteroid sparing while effectively achieving disease control through targeting underlying innate immunity pathways. What is Known: • Idiopathic recurrent pericarditis (IRP) in children is characterized by recurrent inflammatory flares and frequent treatment resistance, with conventional therapies (NSAIDs, colchicine, and corticosteroids) often being insufficient and associated with steroid dependency. • Increasing evidence implicates autoinflammatory pathways in disease pathogenesis, highlighting the potential role of IL-1 inhibition in refractory cases. What is New: • The high prevalence of MEFV-associated variants, frequent steroid dependency, and substantial use of IL-1 inhibitors collectively support an auto inflammatory-driven disease model in pediatric IRP. • Anakinra markedly reduces disease activity even in patients with high baseline inflammatory burden; however, sustained disease control requires continuous therapy, as dose spacing is associated with increased relapse rates.