More severe parotid gland histopathology in paediatric-onset than in adult-onset Sjögren's disease.

Objectives: The aim of this study was to assess the histopathological features of the parotid glands in patients with paediatric-onset Sjögren's disease (pedSjD) in comparison to patients with adult-onset Sjögren's disease (adSjD).

Methods: This study was performed in Groningen, the Netherlands. Patients with pedSjD from a diagnostic paediatric cohort (n=19), patients with adSjD from a diagnostic adult cohort (n=32) and patients with adSjD who participated in a clinical trial (n=42) with a baseline parotid gland biopsy were included.

Clinical Symptoms, Laboratory Parameters and Long-Term Follow-up in a National DADA2 Cohort.

Deficiency of adenosine deaminase-2 (DADA2) is an autosomal recessive autoinflammatory disease with an extremely variable disease presentation. This paper provides a comprehensive overview of the Dutch DADA2 cohort. We performed a retrospective cohort study in 29 ADA2-deficient patients from 23 families with a median age at inclusion of 26 years.

The relation between C-reactive protein and serum amyloid A in patients with autoinflammatory diseases.

Background: Autoinflammatory diseases are rare disorders of the innate immune system characterized by fever and other signs of inflammation. A feared complication of autoinflammatory diseases is the development of AA amyloidosis. AA amyloidosis is caused by extracellular deposition of soluble serum amyloid A (SAA) proteins as insoluble amyloid fibrils leading to organ damage.

Efficacy, Immunogenicity and Safety of Vaccination in Pediatric Patients With Autoimmune Inflammatory Rheumatic Diseases (pedAIIRD): A Systematic Literature Review for the 2021 Update of the EULAR/PRES Recommendations.

Background: In 2011, the first European League Against Rheumatism (EULAR) vaccination recommendations for pediatric patients with autoimmune inflammatory rheumatic diseases (pedAIIRD) were published. The past decade numerous new studies were performed to assess the safety, efficacy and immunogenicity of vaccinations in pedAIIRD. A systematic literature review (SLR) was therefore performed to serve as the basis for the updated 2021 EULAR/PRES recommendations.

EULAR/PRES recommendations for vaccination of paediatric patients with autoimmune inflammatory rheumatic diseases: update 2021.

Objectives: Recent insights supporting the safety of live-attenuated vaccines and novel studies on the immunogenicity of vaccinations in the era of biological disease-modifying antirheumatic drugs in paediatric patients with autoimmune/inflammatory rheumatic diseases (pedAIIRD) necessitated updating the EULAR recommendations.

Methods: Recommendations were developed using the EULAR standard operating procedures. Two international expert committees were formed to update the vaccination recommendations for both paediatric and adult patients with AIIRD.

Differences in presentation between paediatric- and adult-onset primary Sjögren's syndrome patients.

Objectives: Primary Sjögren's syndrome (pSS) is a rare disease in paediatric patients. Presenting symptoms differ from those in adult patients. The aim of this study was to evaluate presenting symptoms, classification criteria and clinical assessments, including salivary gland ultrasonography (SGUS), at disease onset in paediatric and adult patients with pSS.

Neuroinflammatory Disease as an Isolated Manifestation of Hemophagocytic Lymphohistiocytosis.

Isolated neuroinflammatory disease has been described in case reports of familial hemophagocytic lymphohistiocytosis (FHL), but the clinical spectrum of disease manifestations, response to therapy and prognosis remain poorly defined. We combined an international survey with a literature search to identify FHL patients with (i) initial presentation with isolated neurological symptoms; (ii) absence of cytopenia and splenomegaly at presentation; and (iii) systemic HLH features no earlier than 3 months after neurological presentation. Thirty-eight (20 unreported) patients were identified with initial diagnoses including acute demyelinating encephalopathy, leukoencephalopathy, CNS vasculitis, multiple sclerosis, and encephalitis.

Role of FDG-PET/CT in children with fever of unknown origin.

Purpose: To determine the role of F-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET)/computed tomography (CT) in children with fever of unknown origin (FUO).

Methods: This retrospective single-center study included 110 children (0-18 years) with FUO who underwent FDG-PET/CT between 2010 and 2019. The diagnostic value of FDG-PET/CT for identifying cause of fever was calculated, treatment modifications after FDG-PET/CT were assessed, and logistic regression analyses were performed to identify clinical and biochemical factors associated with FDG-PET/CT outcome.

Efficacy of High-Dose Methotrexate in Pediatric Non-Infectious Uveitis.

To analyze the efficacy of high dose (≥ 15mg/m2/week) methotrexate (MTX) versus low dose (<15mg/m2/week) MTX in relation to time to remission on medication. Retrospective observational cohort study of pediatric patients with auto-immune uveitis with or without underlying systemic disease treated with MTX at the University Medical Center Groningen (the Netherlands) between 1990 and 2014. Primary outcome was time to remission on medication, which was defined as an observable inactive disease in the affected eye for longer than 3 months without the use of systemic corticosteroids.

Decreased quality of life and societal impact of cryopyrin-associated periodic syndrome treated with canakinumab: a questionnaire based cohort study.

Background: Cryopyrin-associated periodic syndrome (CAPS) is a rare disease. Knowledge on the quality of life (QoL) and the disease's societal impact is limited. Canakinumab is used in increasing frequency for the treatment of CAPS.

Internet Program for Physical Activity and Exercise Capacity in Children With Juvenile Idiopathic Arthritis: A Multicenter Randomized Controlled Trial.

Objective: To determine the effects of Rheumates@Work, an internet-based program supplemented with 4 group sessions, aimed at improving physical activity, exercise capacity, health-related quality of life (HRQoL), and participation in children with juvenile idiopathic arthritis.

Methods: Patients were recruited from 3 pediatric rheumatology centers in The Netherlands for an observer-blinded, randomized controlled multicenter trial. Physical activity level, time spent in rest, light, and moderate-to-vigorous physical activity (MVPA) were recorded in a diary and with an accelerometer, before intervention, after intervention, and at followup after 3 and 12 months (intervention group only).

[A cavernous haemangioma of the colon as the cause of rectal bleeding in childhood].

A 13-year-old girl presented with rectal bleeding, abdominal pain and a palpable mass in the lower right abdomen. The history mentioned a chronic anaemia since she was 2 years old. Further examination did not give any indications for inflammation as etiological factor and showed a thickening of (apparently) the small intestine.