Spectrum of Central Nervous System Involvement in Childhood-Onset Sjögren's Syndrome: A Case-Based Review.

Sjögren's syndrome (SS) is less frequently diagnosed in childhood than in adults, and central nervous system (CNS) disease is among the rarest systemic involvements. Thus, the clinical spectrum of CNS diseases and their management strategies have not been fully defined. In this article, we present the case of a 16-year-old girl who was referred for severe headache and diagnosed with SS with CNS involvement.

Severe haematological involvement in children with systemic lupus erythematosus and clinical associations.

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.

Evaluation of pediatric rheumatologists' approach to rituximab use: a questionnaire study.

Unlabelled: Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey.

Evaluation of childhood malignancies presenting with musculoskeletal manifestations from two different divisions: a multicenter study.

Background: The aim of the study was to evaluate the approaches of pediatric rheumatologists and pediatric hematologists to patients with similar musculoskeletal (MSK) complaints and to highlight the differences that general pediatricians should consider when referring patients to these specialties.

Methods: This is a cross-sectional study involving the patients who applied to pediatric rheumatology centers with MSK complaints and were diagnosed with malignancy, as well as patients who were followed up in pediatric hematology centers with a malignancy diagnosis, and had MSK complaints at the time of admission.

Results: A total of 142 patients were enrolled in the study.

Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis on anti-interleukin-1 or -6 therapy.

Objectives: The aim of this study is to investigate the effect of anti-interleukin (IL)-1/-6 biologics on systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS).

Methods: Demographic, clinical and laboratory data of patients followed up with a diagnosis of sJIA-associated MAS assessed from sixteen paediatric rheumatology centres across the country. The clinical and laboratory features of MAS developing while on biological drugs were compared with those without this treatment.

Towards a standardized program of transitional care for adolescents with juvenile idiopathic arthritis for Turkey: a national survey study.

Background: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored.

Impact of serological activity on flare following clinically inactive disease and remission in childhood-onset systemic lupus erythematosus.

Objectives: The objectives of this study were to assess the association between serological activity (SA) and clinical inactivity in SLE and to investigate whether SA predicts flare after the attainment of clinically inactive disease (CID) and remission.

Methods: The longitudinal data of children from three paediatric rheumatology referral centres were retrospectively reviewed. CID was interpreted as the beginning of a transitional phase of clinical inactivity on a moderate glucocorticoid dose during which tapering was expected and defined as the absence of disease activity in clinical domains of SLEDAI, without haemolytic anaemia or gastrointestinal activity, in patients using <15 mg/day prednisolone treatment.

The safety of canakinumab in systemic juvenile idiopathic arthritis and autoinflammatory diseases in pediatric patients: a multicenter study.

Objective: To evaluate the safety of canakinumab using real-world data in patients with systemic juvenile idiopathic arthritis (sJIA) and autoinflammatory diseases (AID).

Research Design And Methods: This was a cross-sectional observational, multicenter study. Patients diagnosed with AID and sJIA treated with canakinumab were included in the study.

How common is remission in rheumatoid factor-positive juvenile idiopathic arthritis patients? The multicenter Pediatric Rheumatology Academy (PeRA) research group experience.

Objective: Rheumatoid factor (RF)-positive polyarthritis is the least common type of juvenile idiopathic arthritis (JIA). Functional disability in RF-positive polyarthritis patients is much more severe than in patients with other subtypes; but data on this subtype alone is limited. This study aimed to analyze clinical features, long-term follow-up, treatment response, and remission status in a large multicenter cohort of RF-positive polyarthritis patients.

Letrozole-induced inflammatory arthritis and tendinopathy in pediatric rheumatology setting.

Musculoskeletal symptoms associated with the use of aromatase inhibitors are a well-known side effect of these drugs and are more prevalent in postmenopausal women. Aromatase inhibitor-associated symptoms are not overt inflammatory processes so are described as arthralgia syndrome. In contrast, aromatase inhibitor-associated inflammatory conditions such as myopathies, vasculitis, and rheumatoid arthritis were also reported.

Significance of the weighted complement domain of the EULAR/ACR classification criteria in juvenile onset systemic lupus erythematosus.

Objective: We aimed to compare the clinical and laboratory characteristics of patients with SLE according to the weighted complement status of the EULAR/ACR criteria and investigate whether different weighting of the complement status at disease onset is associated with outcomes.

Methods: Patients diagnosed with juvenile onset SLE who fulfilled the 2019 EULAR/ACR classification criteria were retrospectively analyzed.

Results: Among 43 patients included, hypocomplementemia was observed in 37 (86%), mostly with a low level of both complement C3 (C3) and complement C4 (C4) (53.

Significance of the immunofluorescence staining patterns and titres of the antinuclear antibody test in paediatric rheumatology setting.

Background: Antinuclear antibody (ANA) is among the most frequently ordered tests in paediatric rheumatology setting. Diseases like systemic lupus erythematosus and Sjögren syndrome is closely related with a positive ANA and classified as ANA associated diseases. Besides, ANA test is ordered in patients with juvenile idiopathic arthritis (JIA) to assess the risk for uveitis and a positive ANA could be detected in children with nonrheumaticrheumatic conditions.

Genotype-Phenotype Associations of Children With Familial Mediterranean Fever in a Cohort Consisting of M694V Mutation and Implications for Colchicine-Resistant Disease.

Objectives: The aim of this study was to investigate the clinical associations of the second allele mutations and the effect of genotype and presenting features on colchicine resistance in children with familial Mediterranean fever (FMF), carrying at least one M694V variant.

Methods: The medical records of the patients diagnosed with FMF, in whom at least one allele M694V mutation was detected, were reviewed. Patients were grouped according to the genotype as M694V homozygotes, compound heterozygote M694V with an exon 10 mutation, compound heterozygote M694V with a variant of unknown significance (VUS), and M694V heterozygotes.

Mercury exposure mimicking systemic lupus erythematosus in a thirteen-year-old girl.

Background: The clinical presentation of mercury (Hg) intoxication may mimic rheumatic diseases. Hg exposure is associated with systemic lupus erythematosus (SLE)-like disease in genetically susceptible rodents and Hg is among the environmental factors in the development of SLE in humans. Herein, we presented a case with clinical and immunological features suggestive of SLE but diagnosed with Hg intoxication.

Lupus low disease activity state as a treatment target for pediatric patients with lupus nephritis.

Background: Lupus low disease activity state (LLDAS) is a treatment target for patients with SLE and is associated with decreased risk for severe flare and new damage. We investigated the utility of the achievement of LLDAS in children with lupus nephritis and whether attainment of LLDAS is associated with more favorable outcomes.

Methods: Data of children, diagnosed with biopsy-proven lupus nephritis between January 2012 and December 2020, were retrospectively analyzed.

Depression, anxiety, and sleep quality in childhood onset systemic lupus erythematosus and relationship with brain-derived neurotrophic factor.

Background: The association between brain-derived neurotrophic factor (BDNF) and systemic lupus erythematosus (SLE) is controversial, and no study investigated the clinical associations of BDNF in patients with childhood onset systemic lupus erythematosus (cSLE). In this study, we aimed to investigate the serum levels of BDNF in patients with cSLE and examine whether a relationship of BDNF exists among depression, anxiety, and sleep quality.

Methods: Thirty patients and age-sex matched healthy controls were included.

Hematologic manifestations of juvenile systemic lupus erythematosus: An emphasis on anemia.

Objective: Anemia is common in patients with juvenile systemic lupus erythematosus (jSLE). While autoimmune hemolytic anemia (AIHA) is the only etiology included in the classification criteria, the etiology of anemia in jSLE may be diverse. We aimed to investigate the etiology of anemia in jSLE and the relationship between anemia and disease characteristics at onset and during the follow-up period.