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Objectives: Although Behçet's disease (BD) typically manifests in the second or third decade of life, initial symptoms may appear at a younger age. It may also take a longer time for the full disease phenotype to develop after the first symptom onset in pediatric patients. In this study we aimed to assess the clinical course of pediatric-onset BD in adulthood period., Methods: The files of 112 patients diagnosed with BD before the age of 18, selected from five tertiary clinics, were retrospectively examined. Patients with a follow-up of less than six months were excluded., Results: The study comprised 93 patients with pediatric-onset BD, of whom 64.5% (n = 60) were male. The median age of diagnosis was 15 years (13-17). Major organ involvement was present in 49 (52.5%) patients. The most commonly affected organ was the eye (29%).Sixty-eight (73.1%) patients had follow-up data in adulthood. Fourty patients had only mucocutaneous manifestations in the pediatric period. During follow-up in adulthood, 15 (53.3% were male) had new major organ involvement with a mean of 10.1 (SD: 7.9) years after diagnosis. Twenty-eight patients (41.1%) experienced major organ involvement during the pediatric period. In adulthood follow-up, 12 (42.8%) developed new major organ involvement and/or relapse of the same organ. Eighteen (26.5%) of 68 pediatric-onset BD patients had new major organ involvement, and 9 (13.2%) had a relapse during adulthood follow-up., Conclusion: Our results showed that nearly one-third of pediatric BD patients have a new major organ involvement or a relapse in adulthood. Regular follow-up of pediatric BD patients in adulthood is essential to prevent long-term damage in this disease subset., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

To evaluate the sleep quality and fatigue levels in children with familial Mediterranean fever (FMF) in comparison to healthy children. The Pediatric Quality of Life Multidimensional Fatigue Scale (PedsQL-MFS) and the Pittsburgh Sleep Quality Index (PSQI) were the instruments utilized to assess fatigue and sleep quality in children with FMF and controls, respectively. Spearman's rank coefficient was decisive in determining the association between patient-reported outcome measures and disease-related features. Two hundred twenty-five (59.3% female) patients and 182 (51.6% female) healthy counterparts were enrolled in the study. In PSQI, where high scores indicate sleep disturbance, the median score was significantly higher in the patient group (5; 3-6) than the control group (3; 2-4) (p < 0.001). PEDsQL-MFS demonstrated significantly lower fatigue levels in the control group than patients (p = 0.01). The level of fatigue in the patient group was found to increase in correlation with sleep problems (r: - 0.750, p < 0.001). Additionally, a high correlation was present between the PSQI/PedsQL-MFS scores and the number of attacks in the last year (r: - 0.645, p < 0.001/r: 0.721, p < 0.001, respectively). There was no difference in terms of fatigue and sleep disorders between mutations (homozygous, heterozygous, or compound heterozygous) in the MEFV gene (p > 0.05).    Conclusion: High disease activity has a significant negative impact on the sleep quality and fatigue levels of patients with FMF. This study emphasizes the importance of assessing fatigue and sleep quality with objective outcome tools periodically in FMF patients throughout the disease course. What is Known: • Fatigue is a common matter that often accompanies rheumatic diseases and causes disability. • Chronic rheumatic diseases often experience poor sleep quality. What is New: • In high correlation with the disease severity of familial Mediterranean fever, sleep quality decreases and fatigue level increases significantly. • In familial Mediterranean fever patients, a negative correlation is present between age and the general fatigue and sleep/rest related fatigue scores (low scores indicating greater fatigue) and sleep quality is poorer in the adolescent age group., (© 2024. The Author(s).)

Objectives: This study aimed to explore the influence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic among patients with juvenile systemic sclerosis (JSS)., Patients and Methods: Twenty-seven patients (22 females, 5 males; mean age: 20 years; range, 17 to 22 years) diagnosed with JSS and followed up at the department of pediatric rheumatology were included in the cross-sectional study. A web-based survey was performed by focusing on patients' complaints, accessibility to health care, and compliance with routine treatment from January 1, 2021, to January 10, 2021., Results: Five (18.5%) patients had deterioration of the disease, while six (22.2%) patients reported irregular usage of their routine scleroderma treatment during the last six months. Nine (33.3%) patients had missed their routine clinic control since the proclamation of the SARS-CoV-2 pandemic. Seven (25.9%) patients had household contact with coronavirus disease 2019 (COVID-19). Four (14.8%) patients were diagnosed with COVID-19, and only one (3.7%) was hospitalized. Nine patients were under biological treatment (tocilizumab); however, only one of them was diagnosed with COVID-19., Conclusion: The COVID-19 pandemic has not significantly disrupted the medical care of JSS patients. Telemedicine could be an acceptable option for JSS patients disenabled to come to the hospital., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2023, Turkish League Against Rheumatism.)

Background: Juvenile systemic sclerosis (JSS) is an extremely rarely seen auto-immune disease characterized by the increased fibrosis of skin and internal organs. Congenital pulmonary airway malformation (CPAM) is a developmental disorder of the lung, characterized by atypical cell hyperplasia which creates the ground for lung adenocarcinoma. In general, CPAM is diagnosed in early childhood, due to recurrent respiratory symptoms including cough, hemoptysis and respiratory infections. Although rare, there are some sporadic asymptomatic cases of CPAM that have been reported. We present a case with a coincidental presence of two rare diseases: JSS and CPAM., Case: An adolescent female patient was admitted to hospital due to clinical signs of JSS. During the followup, the patient had been diagnosed with cystic adenoid malformation of the lung complicated by mucinous adenocarcinoma. The patient was previously healthy with an unremarkable history, including lack of respiratory symptoms. Left inferior lobectomy was performed. Considering the small size of malignant loci, the total resection of the tumor and absence of any sign for metastasis disease, adjuvant therapy was not scheduled. We haven`t found a pediatric case of CPAM associated adenocarcinoma of the lung presented by signs of JSS in the literature. In this case, the clinical signs of JSS possibly represent part of the paraneoplastic syndrome related to adenocarcinoma of the lung., Conclusions: Internal organ involvement, including respiratory system, should not be omitted even in asymptomatic patients with JSS. Auto-antibody negativity represents a clue for the possible underlying condition. Further studies with a higher number of patients would reveal more relevant data.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The disease is divided in different subtypes based on main clinical features and disease course. Emergence of biological agents targeting specific pro-inflammatory cytokines responsible for the disease pathogenesis represents the revolution in the JIA treatment. Discovery and widespread usage of biological agents have led to significant improvement in JIA patients' treatment, with evidently increased functionality and decreased disease sequel. Increased risk of infections remains the main discussion topic for years. Despite the slightly increased frequency of upper respiratory tract infections reported in some studies, the general safety of drugs is acceptable with rare reports of severe adverse effects (SAEs). Tuberculosis (TBC) represents the important threat in regions with increased TBC prevalence. Therefore, routine screening for TBC should not be neglected when prescribing and during the follow-up of biological treatment. Malignancy represents a hypothetical complication that sometimes causes hesitations for physicians and patients in its prescription and usage. On the other hand, current reports from the literature do not support the increased risk for malignancy among JIA patients treated with biological agents. A multidisciplinary approach including a pediatric rheumatologist and an infectious disease specialist is mandatory in the follow- up of JIA patients. Although the efficacy and safety of biological agents have been proven in different studies, there is still a need for long-term, multicentric evaluation providing relevant data., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2020, Turkish League Against Rheumatism.)

Behçet's Disease was first described by a Turkish dermatologist, Hulusi Behçet, in 1937 as a triple symptom complex; aphthous stomatitis, genital ulcers, and uveitis. Today, in light of current trials and experiments, we know that the disease may have a wider involvement with a multisystemic recurrent course, causing significant morbidity and mortality. However, there are still unanswered questions, particularly about Pediatric Behçet's Disease. Although several immunological and genetic associations have been demonstrated, the real etiologic mechanism of the disease is unclear. The diagnosis is difficult due to its rarity in childhood, the lack of validation of the diagnostic criteria obtained from adult studies, and the inadequacy of large case-controlled studies. Also, the management is challenging and controversial due to the various geographic distribution of clinical spectrum. New therapeutic options under development in light of pathogenetic hypothesis seem to be promising.

Köker O, Yıldırmak ZY, Genç DB, Kılıçaslan Ö. Thrombotic thrombocytopenic purpura as a rare cause of anemia with thrombocytopenia in childhood: report of 2 cases. Turk J Pediatr 2019; 61: 418-423. Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem disorder characterized by single or recurrent episodes of thrombocytopenia, microangiopathic hemolytic anemia and widespread microvascular thrombosis, which causes significant morbidity and mortality unless promptly recognized and treated. The underlying pathogenesis is a defect in von Willebrand factor (vWF) cleaving protease, called `A Disintegrin and Metalloproteinase with Thrombospondin Type 1 Repeats 13 (ADAMTS-13)`. There are 2 forms: congenital TTP (ADAMTS-13 gene mutations) and acquired TTP (autoantibodies and ADAMTS-13 deficiency). We presented two patients who initially presented with thrombotic microangiopathy and were later diagnosed with TTP upon demonstration of the deficiency in ADAMTS-13 activity.

The effect of immunotherapy on 21 Dermatophagoides pteronyssinus (Dpt)-sensitive patients (15 children with extrinsic asthma and six children with extrinsic asthma plus allergic rhinitis) aged 8.9 +/- 0.71 years was studied. Their subjective and objective respiratory symptom score was recorded before beginning and after the first year of immunotherapy. The respiratory subjective symptoms, such as cough day/night, running nose, sputum production, sneezing, shortness of breath at night and asthma attacks, decreased significantly from pretreatment level (P < 0.05). The objective symptoms, such as wheezing, dyspnea with prolonged expiration, sibilant rales and retractions score, gradually decreased during treatment (P < 0.01). Total eosinophil count decreased in 76.2% of patients after immunotherapy (P < 0.01). While total immunoglobulin E (IgE) decreased in 81% of patients and specific IgE for Dpt decreased in 90.5% of patients, specific IgG for Dpt increased in 76% of patient (P < 0.001). Our results suggested that rising IgG and falling IgE values do not always coexist in the patients who benefit from immunotherapy.