Validity of an ultrasonographic joint-specific scoring system in juvenile idiopathic arthritis: a cross-sectional study comparing ultrasound findings of synovitis with whole-body magnetic resonance imaging and clinical assessment.

Objective: To assess the validity of an ultrasonographic scoring system in juvenile idiopathic arthritis (JIA) by comparing ultrasound detected synovitis with whole-body MRI and clinical assessment of disease activity.

Methods: In a cross-sectional study, 27 patients with active JIA underwent clinical 71-joints examination, non-contrast enhanced whole-body MRI and ultrasound evaluation of 28 joints (elbow, radiocarpal, midcarpal, metacarpophalangeal 2-3, proximal interphalangeal 2-3, hip, knee, tibiotalar, talonavicular, subtalar and metatarsophalangeal 2-3). One rheumatologist, blinded to clinical findings, performed ultrasound and scored synovitis (B-mode and power Doppler) findings using a semiquantitative joint-specific scoring system for synovitis in JIA.

Persisting mortality gap in systemic lupus erythematosus; a population-based study on juvenile- and adult-onset SLE in Norway 1999-2022.

Objective: To estimate mortality and survival rates of SLE in a contemporary, population-based setting and assess potential influences by time, sex, ethnicity, classification criteria and age at diagnosis.

Methods: We assessed mortality and survival in the Nor-SLE cohort, which includes all chart review-confirmed SLE cases resident in Southeast Norway (population 2.9 million) 1999-2017.

Associations between power Doppler ultrasound findings and B-mode synovitis and clinical arthritis in juvenile idiopathic arthritis using a standardised scanning approach and scoring system.

Objectives: To describe power Doppler (PD) ultrasound findings in joint regions with B-mode (BM) synovitis using a standardised scanning protocol and scoring system in patients with juvenile idiopathic arthritis (JIA). Further, to examine associations between PD findings and BM synovitis, clinical arthritis, patient characteristics and disease activity.

Methods: In this cross-sectional study, one experienced ultrasonographer, blinded to clinical findings, performed ultrasound examinations in 27 JIA patients with suspected clinical arthritis.

Cardiac dysfunction in mixed connective tissue disease: a nationwide observational study.

We aimed to identify cardiac function in patients with established mixed connective tissue disease (MCTD). This was a cross-sectional case-control study of well-characterised MCTD patients who had previously been included in a nationwide cohort. Assessments comprised protocol transthoracic echocardiography, electrocardiogram and blood samples.

Development and reliability of a novel ultrasonographic joint-specific scoring system for synovitis with reference atlas for patients with juvenile idiopathic arthritis.

Objective: To develop an ultrasonographic image acquisition protocol and a joint-specific scoring system for synovitis with reference atlas in patients with juvenile idiopathic arthritis (JIA) and to assess the reliability of the system.

Methods: Seven rheumatologists with extensive ultrasound experience developed a scanning protocol and a semiquantitative joint-specific scoring system for B-mode (BM) synovitis for the elbow, wrist, metacarpophalangeal 2-3, proximal interphalangeal 2-3, hip, knee, ankle and metatarsophalangeal 2-3 joints. An ultrasonographic reference atlas for BM synovitis, divided in four age groups (2-4, 5-8, 9-12, 13-18 years), and power Doppler (PD) activity was then developed.

Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases.

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.

Increased Endothelial Activation in Patients with Mixed Connective Tissue Disease.

Objective: Juvenile-onset mixed connective tissue disease (JMCTD) is a chronic inflammatory disease. We have previously demonstrated preclinical atherosclerosis in these patients, now exploring this further by assessing markers of endothelial dysfunction.

Methods: Thirty-three patients with JMCTD and 33 age-and sex-matched controls were included.

Effect on Cardiac Function of Longstanding Juvenile-onset Mixed Connective Tissue Disease: A Controlled Study.

Objective: To assess cardiac function in patients with juvenile mixed connective tissue disease (JMCTD) compared to matched controls, and to investigate possible associations between cardiac impairment and disease variables and cardiovascular risk factors.

Methods: Fifty JMCTD patients (86% female) examined median 14.9 (6.

Preclinical Carotid Atherosclerosis in Patients With Juvenile-Onset Mixed Connective Tissue Disease.

Background: This study investigated preclinical atherosclerosis in patients with juvenile mixed connective tissue disease (JMCTD), which is a chronic inflammatory disease with a varied phenotype. Mixed connective tissue disease (MCTD) has well known associations with other autoimmune diseases known to have increased risk of cardiovascular disease. However, the cardiovascular risk for patients with the juvenile form remains unclear.

Juvenile Sjögren's Syndrome: Clinical Characteristics With Focus on Salivary Gland Ultrasonography.

Objective: Juvenile Sjögren's syndrome (SS) is a rare, poorly defined, and possibly underdiagnosed condition affecting children and adolescents. The aim of this study was to characterize symptoms and clinical findings of juvenile SS and to explore the clinical application of major salivary gland ultrasonography (SGUS) in patients with juvenile SS.

Methods: A cross-sectional multicenter study recruited patients with disease onset until age 18 years (n = 67).

Arterial properties in adults with long-lasting active juvenile idiopathic arthritis compared to healthy controls.

Background: The data on cardiovascular risk and systemic arterial properties in patients with long-lasting juvenile idiopathic arthritis (JIA) is limited. The objective of this study was to describe systemic arterial properties including characteristic impedance (Z), total arterial compliance (C), and peripheral vascular resistance (R) in patients with long-lasting active JIA compared with matched controls, and to assess the relation to JIA disease variables and traditional cardiovascular risk factors.

Findings: Methods: Eighty-one JIA patients (median age 38.

Pulmonary Manifestations and Progression of Lung Disease in Juvenile-onset Mixed Connective Tissue Disease.

Objective: To assess the occurrence and extent of interstitial lung disease (ILD) in patients with juvenile mixed connective tissue disease (JMCTD), compare pulmonary function in patients and matched controls, study associations between ILD and disease-related variables, and examine progression of pulmonary manifestations over time.

Methods: A cohort of 52 patients with JMCTD were examined in a cross-sectional study after a mean 16.2 (SD 10.

Physical Functioning, Pain, and Health-Related Quality of Life in Adults With Juvenile Idiopathic Arthritis: A Longitudinal 30-Year Followup Study.

Objective: To describe physical functioning, pain, and health-related quality of life (HRQoL) in adults with juvenile idiopathic arthritis (JIA), investigate changes over time, and identify predictors of poorer HRQoL after 30 years of disease.

Methods: Patients (n = 176) clinically examined after 15 years were reassessed using the Health Assessment Questionnaire disability index (HAQ DI), the visual analog scale pain subscale (VAS pain), and the Medical Outcomes Study Short Form 36 (SF-36) after 23 years and 30 years. Patients with signs of active disease after a minimum of 15 years were clinically examined again at 30 years.

Radiographic damage in hands and wrists of patients with juvenile idiopathic arthritis after 29 years of disease duration.

Background: There are few studies on radiographic outcome after long-term disease duration in juvenile idiopathic arthritis (JIA). We wanted to evaluate 29-year radiographic outcome in hands/wrists and predictors of damage in patients with long-term active JIA.

Methods: Patients diagnosed from 1980 to 1985, who had active disease at 15-, 23- or 29-year follow-up and arthritis in the wrists during the disease course, were reexamined with radiographs of hands/wrists.

Stroke as the Sole Manifestation of Takayasu Arteritis in a 15-Year-Old Boy with Latent Tuberculosis.

. Takayasu arteritis is a rare disease affecting the aorta and its main branches, causing arterial claudication and end-organ ischemia, including stroke. The etiology is unknown but is believed to be autoimmune.

Mixed connective tissue disease.

The concept of mixed connective tissue disease (MCTD) as a separate connective tissue disease (CTD) has persisted for more than four decades. High titers of antibodies targeting the U1 small nuclear ribonucleoprotein particle (U1 snRNP) in peripheral blood are a sine qua non for the diagnosis of MCTD, in addition to distinct clinical features including Raynaud's phenomenon (RP), "puffy hands," arthritis, myositis, pleuritis, pericarditis, interstitial lung disease (ILD), and pulmonary hypertension (PH). Recently, population-based epidemiology data from Norway estimated the point prevalence of adult-onset MCTD to be 3.

Long-term outcome in juvenile-onset mixed connective tissue disease: a nationwide Norwegian study.

Objectives: To describe the characteristics, outcome and predictive factors of juvenile mixed connective tissue disease (JMCTD) in a nationwide cohort of patients.

Methods: We examined 55 patients with JMCTD after a mean disease duration of 16.2 years (SD 10.

Cardiac Function in Adult Patients with Juvenile Idiopathic Arthritis.

Objective: To compare cardiac function in adults with longterm juvenile idiopathic arthritis (JIA) with that of healthy controls, and to investigate the influence of inflammation, disease severity, and use of antirheumatic medication on cardiac function.

Methods: Eighty-five patients with JIA (median age 38.6 yrs) with active disease for at least 15 years were reexamined at a median of 29 years after disease onset and compared with 46 matched controls.

Disease progression into adulthood and predictors of long-term active disease in juvenile idiopathic arthritis.

Objectives: To describe disease activity 30 years after disease onset in a previously studied cohort of patients with juvenile idiopathic arthritis (JIA) and reveal predictors of long-term active disease.

Methods: Patients with JIA, first referred 1980-1985 and re-examined 15 and 23 years after onset, were invited to attend. All 176 patients were assessed by questionnaires.

Arterial haemodynamics and coronary artery calcification in adult patients with juvenile idiopathic arthritis.

Objective: To compare arterial haemodynamics in adults with long-term juvenile idiopathic arthritis (JIA) to that of healthy controls, and explore the influence of traditional cardiovascular risk factors and disease characteristics on arterial haemodynamics plus coronary artery calcification.

Methods: 87 JIA patients (median age 38.4 years) with persistently active disease at least 15 years after disease onset (registered by longitudinal follow-up), were re-examined after median 29 years and compared with 87 matched controls.