Nihal Şahin

The purpose of the study is to evaluate the demographic characteristics, clinical features, and management strategies of TNF inhibitor-related autoimmune d

Objective: FMF is highly prevalent in Mediterranean countries and represents a substantial proportion of paediatric rheumatology patients. Colchicine, the mainstay of treatment, requires lifelong daily use, yet long-term adherence remains challenging, leading to recurrent attacks and increased healthcare burden. This study aimed to develop a freely downloadable mobile application to improve medication adherence by providing reminders and enabling direct communication with physicians. Methods: The application was designed as a structured medication-timing and monitoring tool, providing time-specific medication reminders, real-time intake confirmation (‘taken/missed’) and digital adherence tracking. The user interface was tailored to the needs of patients and parents, considering children’s attention span, parental workload and health literacy. Following pilot testing in seven volunteer patients, the application was refined based on user feedback. Physicians used a web-based panel to register patients, define prescriptions and treatment schedules, and assign calendar-based appointments. Medication adherence was assessed using the MASIF scale, and disease activity was evaluated using the AIDAI score. A total of 93 patients with FMF were included in the study. Results: Before application use, adherence was low, with a median MASIF score of 44 and most patients demonstrating poor adherence. After implementation, MASIF scores significantly improved at 3, 6 and 12 months, with a marked reduction in poorly adherent patients (P < 0.001). AIDAI scores and attack frequency significantly decreased, and no patient had an AIDAI score >9 during follow-up. Agreement between application-measured adherence and MASIF scores was excellent (ICC > 0.90). Conclusion: Mobile reminder applications are low-cost, accessible digital health tools with strong potential to enhance medication adherence in FMF patients.

Introduction: Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in childhood and exhibits a heterogeneous disease course. This study aimed to analyse and identify phenotypic clusters by examining the laboratory findings, nailfold capillaroscopy results, and myositis- specific autoantibodies (MSAs) in patients with JDM. Material and methods: This retrospective cohort study included data from patients with JDM treated at the Paediatric Rheumatology Departments of 14 advanced health centres in Turkey. A categorical principal component analysis (CATPCA)-based hierarchical cluster analysis method was employed for clustering. Results: A total of 176 JDM patients were enrolled, and 5 phenotypic clusters were identified using 23 categorical variables. These clusters were interpreted as follows: Cluster A with severe muscle weakness and oesophageal involvement requiring intensive immunosuppressive treatment; Cluster B with amyopathic/hypomyopathic patients; Cluster C with skin manifestations and lung involvement; Cluster D with complicated skin manifestations; and Cluster E with classic JDM. The clinical and laboratory findings and treatments of these 5 clusters were compared. Fatigue, myalgia, photosensiti­vity, Raynaud’s phenomenon, and the use of pulse glucocorticosteroids, intravenous immuno­globulin, and cyclophosphamide treatments differed between the groups (p < 0.001, p = 0.002, p = 0.015, p = 0.036, p = 0.002, p = 0.006, and p = 0.024, respectively). Myositis-specific autoantibodies results were available for 119 patients (65.3%). The most frequent MSAs were antinuclear matrix protein 2 (26.1%) and anti-transcription intermediary factor 1 (20.9%). However, no significant differences were found in MSAs or nailfold capillaroscopy findings. Conclusions: We identified 5 clusters based on patient symptoms and findings. The identification of these 5 clusters can guide more effective treatment strategies in clinical practice. Additionally, these approaches may contribute to improving patients’ quality of life and long-term outcomes by increasing the feasibility of individualised treatment.

Objectives: Behçet’s disease (BD) is a multisystemic inflammatory disease, and its neurologic involvement represents serious manifestations. This study aimed to elucidate the neurologic manifestations of BD in a large paediatric population and to describe its outcomes and prognosis. Methods: This retrospective multicentre study included 75 paediatric patients diagnosed with neuro-Behçet’s disease (NBD). Demographic data, clinical features, neuroimaging findings, treatments and disability conditions were reviewed. Results: Neurologic manifestations were observed in 52 patients (69.3%) at initial presentation and in 23 patients (30.7%) at a median of 14 months (8–30) after diagnosis. The distribution of NBD subgroups was as follows: 56 patients (74.7%) with nonparenchymal, 15 patients (20%) with parenchymal and 4 patients (5.3%) with mixed forms. Headache, observed in 70 patients (93.3%), was the most common neurological symptom, being significantly more frequent in the non-parenchymal form (P = 0.028). Paresis, sensory loss and mood/mental health problems were significantly more frequent in the parenchymal form (P = 0.034, P = 0.016, P = 0.005, respectively). Neuroimaging revealed parenchymal lesions in 19 patients (25.3%), and vascular lesions were in 55 patients (73.3%). Azathioprine was the most commonly used immunosuppressor therapy (78.7%). The median follow-up duration of patients was 30 months (17.5–52.5), during which significant clinical improvement was observed, with no deaths reported. Conclusion: Paediatric NBD remains a significant issue in paediatric rheumatology. The non-parenchymal subgroup is the predominant form in children. Paediatric NBD should be confirmed and classified by MRI. The early aggressive immunosuppressive therapy provides clinical improvement. Activity and disability indexes are valuable for treatment and follow-up.

Background/Aims: The primary objective of this investigation is to systematically assess the resemblances and distinctions between enthesitisrelated arthritis (ERA), which serves as a pediatric analog to ankylosing spondylitis (AS), and AS. Materials and Methods: A cross-sectional analysis was conducted within a pediatric rheumatology facility and an adult rheumatology institution. Demographic and clinical attributes were juxtaposed between individuals diagnosed with AS and those with ERA. Results: A cumulative total of 200 subjects (101 minors, 99 adults) were scrutinized. The prevalence of peripheral arthritis was significantly elevated in ERA (54.5%) compared to AS (21.2%, P < .001), whereas sacroiliitis, axial involvement, and morning stiffness were more prevalent in AS. Nonsteroidal anti-inflammatory drugs constituted the predominant initial therapeutic approach (99% vs. 91.9%, P = .01). Patients with ERA exhibited a higher utilization of corticosteroids (35.6% vs. 11.1%, P < .001) and diseasemodifying antirheumatic drugs (DMARDs) (87.1% vs. 60.6%, P < .001), with no notable variance in the application of biological agents. At the final follow-up assessment, 24.8% of ERA patients were free from medication, whereas no AS patients achieved a medication-free status. Conclusion: An increased volume of scientific evidence is requisite concerning the attributes of ERA, which is presently regarded predominantly within the framework of juvenile spondyloarthritis, distinguishing it from its adult counterpart; this study serves as an observational cohort investigation that underscores this imperative. Despite the observable differences and similarities between ERA and AS, further extensive studies incorporating inflammatory biomarkers are essential to ascertain whether these conditions represent discrete diseases or exist within a broader disease continuum. Cite this article as: Unan MK, Özaslan Z, Dilek G, Şahin N, Sönmez HE, Nas K. Juvenile vs. adult-onset spondyloarthritis: Uncovering similarities and differences. Arch Rheumatol. 2026;41(2):90-99.

The primary objective of this investigation is to systematically assess the resemblances and distinctions between enthesitisrelated arthritis (ERA), which serves as a pediatric analog to ankylosing spondylitis (AS), and AS. A cross-sectional analysis was conducted within a pediatric rheumatology facility and an adult rheumatology institution. Demographic and clinical attributes were juxtaposed between individuals diagnosed with AS and those with ERA. A cumulative total of 200 subjects (101 minors, 99 adults) were scrutinized. The prevalence of peripheral arthritis was significantly elevated in ERA (54.5%) compared to AS (21.2%, P < .001), whereas sacroiliitis, axial involvement, and morning stiffness were more prevalent in AS. Nonsteroidal anti-inflammatory drugs constituted the predominant initial therapeutic approach (99% vs. 91.9%, P = .01). Patients with ERA exhibited a higher utilization of corticosteroids (35.6% vs. 11.1%, P < .001) and diseasemodifying antirheumatic drugs (DMARDs) (87.1% vs. 60.6%, P < .001), with no notable variance in the application of biological agents. At the final follow-up assessment, 24.8% of ERA patients were free from medication, whereas no AS patients achieved a medication-free status. An increased volume of scientific evidence is requisite concerning the attributes of ERA, which is presently regarded predominantly within the framework of juvenile spondyloarthritis, distinguishing it from its adult counterpart; this study serves as an observational cohort investigation that underscores this imperative. Despite the observable differences and similarities between ERA and AS, further extensive studies incorporating inflammatory biomarkers are essential to ascertain whether these conditions represent discrete diseases or exist within a broader disease continuum. Cite this article as: Unan MK, Özaslan Z, Dilek G, Şahin N, Sönmez HE, Nas K. Juvenile vs. adult-onset spondyloarthritis: Uncovering similarities and differences. Arch Rheumatol. 2026;41(2):90-99.

Objectives: Biologic disease-modifying antirheumatic drugs (bDMARDs) have been frequently used to treat juvenile idiopathic arthritis (JIA) resistant to classical (c) DMARDs. There is concern that vaccines may reduce vaccine effectiveness due to their immunosuppressive effects. This study aimed to evaluate the prevalence of anti-hepatitis B surface (HBs) antibody positivity in JIA patients treated with bDMARDs and to compare it with that in JIA patients treated with cDMARDs and in healthy controls. Materials and Methods: Anti-HBs antibody positivity and titers were compared between patients with JIA treated with bDMARDs or only cDMARDs, followed in our clinic, and healthy controls aged 2-20 years. All participants were vaccinated in infancy according to the routine vaccination schedule in our country. Anti-HBs titers ≥10 IU/L were considered seroprotective. Results: Ninety-two JIA patients receiving cDMARDs, 92 receiving bDMARDs, and 91 healthy controls were included in the study. The median time from the last vaccination to the study was 12.5 (min-max:3.5-17.55) years in controls, 12.5 (min-max:1.75-19.5) years in patients with cDMARDs, and 13.0 (min-max:2.25-19.0) years in patients with bDMARDs (p=0.060). Anti-HBs was positive in 50 (54.9%) controls, 66 (71.7%) patients with cDMARDs, and 62 (67.4%) patients with bDMARDs (p=0.048). Age, time from vaccination to study, and duration of bDMARD use were found to be risk factors for anti-HBs negativity in univariate regression analyses. According to the multivariable analysis of these three variables, the duration of bDMARD use was an independent risk factor for anti-HBs negativity (OR: 1.023, 95% CI: 1.005-1.041, p=0.013). Anti-HBs negativity was associated with a duration of bDMARDs longer than 32 months (AUC: 0.658, 95%CI: 0.541-0.776, p=0.014) with 60.0% sensitivity, and 59.7% specificity. Conclusions: A longer duration of bDMARDs was found to be a risk factor for anti-HBs negativity. Physicians should be careful in terms of anti-HBs negativity when the duration of biologics use is prolonged.

Introduction: Juvenile spondyloarthropathies (JSpA) are a group of chronic inflammatory diseases that differ in their clinical features and course from adult spondyloarthropathies and other subtypes of juvenile idiopathic arthritis (JIA). Therefore, defining disease inactivity in JSpA requires specific criteria. This Delphi study aimed to establish a national consensus on its core clinical, laboratory, and radiological domains. Methods: A total of 27 pediatric rheumatologists participated in the Delphi survey, conducted in two rounds. Participants were asked multiple‐choice and Likert‐type questions regarding their preferences for using domains including anamnesis, laboratory findings, imaging methods, and predefined disease activity scores for assessing inactivity. At the end of each round, the study coordinators determined the “strong consensus” items based on a power analysis of these parameters. Results: The absence of “pain or tenderness in the peripheral joints, lower back and entheseal regions” in anamnesis domain and “tenderness in the peripheral joints, entheseal areas, and hip examination”; “swelling in the peripheral joints; tenderness on sacroiliac compression testing”; and “no reduction in hip RoM examination” in physical examination domain received the highest scores and were accepted as strong consensus. Furthermore, normalization of MRI findings of SIJ and hip/peripheral joint and “physician global score = 0” reached the specified thresholds, resulting in strong consensus following the second round. Conclusions: This Delphi study highlights the need for a multidimensional approach that integrates clinical, radiological, and physician assessments to define disease inactivity in patients with JSpA. The resulting consensus provides a more specific assessment on inactivity defining JSpA patients and reflects a national consensus.

Amaç: Eklem ağrısı çocuklarda sık görülen bir yakınma olup, çoğunlukla iyi huylu veya romatolojik olmayan nedenlere bağlıdır. Romatolojik hastalıklar daha nadir saptanır. Bu çalışmada, üçüncü basamak bir hastanenin çocuk romatoloji polikliniğine eklem ağrısı şikayeti ile başvuran hastalarda altta yatan nedenleri belirlemek ve hangi klinik özelliklerin ileri inceleme gereksinimini öngördüğünü saptamak amaçlanmıştır. Yöntem: Bu çalışmada 01.07.2022-01.01.2023 tarihleri arasında çocuk romatoloji polikliniğine eklem ağrısı şikayeti ile yönlendirilen hastalar geriye dönük olarak değerlendirildi. Demografik, klinik ve laboratuvar özellikleri hasta dosyalarından incelendi. Bulgular: Çalışmaya dahil edilen 254 hastanın %57,1’i kız ve yaş ortalaması 10,5±4,6 yıldı. Eklem ağrısı şikayetinin başlangıç yaşı ortanca 10 (5,3-13,5) yıl ve ortanca şikayet süresi 6 (3-12) aydı. Çocukların 115 (%45,3)’ünde patoloji saptanmazken, 20 (%7,9)’unda romatolojik hastalık, 119 (%46,9)’unda non-romatolojik hastalık tanısı konuldu. Romatolojik hastalığı olan çocukların şikayet süresi daha uzundu (medyan 9 ay [2,4–21]) (p<0,001) ve devamlı ağrı bildirme oranı %75 ile anlamlı şekilde yüksekti (p=0,001). Bu grupta ağrının aktiviteyle azalması (%40, p<0,001), istirahat ile gerilememesi (%40 istirahate yanıtlı, p<0,001), gece uyandıran ağrı (%10, p=0,007) ve masaja yanıt vermeyen ağrı (%5, p=0,01) gibi inflamatuvar özellikler belirgin olarak daha fazlaydı. Sonuç: Çocuk romatoloji polikliniğine eklem ağrısı şikayeti ile başvuranların az bir kısmı romatolojik hastalık tanısı almıştır. Romatolojik hastalığı olan çocuklarda eklem ağrısı genellikle daha uzun süreli ve sürekli olup, ağrı karakteri çoğunlukla inflamatuvar özellik göstermektedir. Eklem ağrısı şikayeti olan çocuklarda dikkatli anamnez ve fizik muayene ile gereksiz ileri tetkik ve yönlendirmeler önlenebilir.