Clinical features of childhood localized scleroderma in an incidence cohort.

Rheumatology (Oxford)

Arthritis Research UK Epidemiology Unit, University of Manchester, Stopford Building, Oxford Road, Manchester M13 9PT, UK.

Published: October 2011

Objectives: Our aim was to describe clinical features and pattern of care in children with localized scleroderma presenting to secondary care during a 25-month incidence study.

Methods: Eighty-seven patients were identified, and clinical features, serum autoantibodies, current treatment and outcome at 12 months were documented.

Results: Fifty-eight (67%) had linear scleroderma, 25 (29%) non-linear morphoea and 4 (4%) a mixed pattern. Of the 58 patients with linear scleroderma, 29 (50%) presented with lesions of the trunk and/or limbs only, 26 (45%) with face-head localization only and 3 (5%) with both. Thirteen (15%) had extracutaneous features and 16 (43%) out of 37 were ANA positive. At 12 months, 59% were on MTX. At 12 months, 51 (65%) were improved/resolved, 14 (18%) were unchanged and 13 (17%) had deteriorated.

Conclusion: Key findings included the high prevalence of face-head involvement in those with linear disease, and the high prevalence of extracutaneous disease and of ANA positivity. After 12 months, most patients improved according to clinician's opinion.

Download full-text PDF

Source
http://dx.doi.org/10.1093/rheumatology/ker142DOI Listing

Publication Analysis

Top Keywords

clinical features
12
localized scleroderma
8
linear scleroderma
8
high prevalence
8
features childhood
4
childhood localized
4
scleroderma
4
scleroderma incidence
4
incidence cohort
4
cohort objectives
4

Similar Publications

Comprehensive histopathological analysis of gastric cancer in European and Latin America populations reveals differences in PDL1, HER2, p53 and MUC6 expression.

Gastric Cancer

January 2025

Department of Medical Oncology, Hospital Clinico Universitario, INCLIVA, Biomedical Research Institute, University of Valencia, Avenida Menendez Pelayo nro 4 accesorio, Valencia, Spain.

Introduction: Gastric cancer (GC) burden is currently evolving with regional differences associated with complex behavioural, environmental, and genetic risk factors. The LEGACy study is a Horizon 2020-funded multi-institutional research project conducted prospectively to provide comprehensive data on the tumour biological characteristics of gastroesophageal cancer from European and LATAM countries.

Material And Methods: Treatment-naïve advanced gastroesophageal adenocarcinoma patients were prospectively recruited in seven European and LATAM countries.

View Article and Find Full Text PDF

A Randomized, Controlled Trial of In-Hospital Use of Virtual Reality to Reduce Preoperative Anxiety Prior to Cardiac Surgery.

Mayo Clin Proc

January 2025

Departments of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA; Physiology and Biomedical Engineering, Mayo Clinic, Rochester, MN, USA; Robert and Arlene Kogod Center on Aging, Mayo Clinic, Rochester, MN, USA. Electronic address:

Objective: To study the effectiveness of virtual reality (VR) in reducing anxiety levels in patients undergoing first-time sternotomy for cardiac surgery.

Patients And Methods: A total of 100 adult patients scheduled for cardiac surgery at Mayo Clinic in Rochester, Minnesota, USA, was recruited from April 19, 2022, to October 12, 2022. Before surgery, patients wore a physiological monitor to record vital signs.

View Article and Find Full Text PDF

Introduction: Pain is one of the most frequently reported symptoms in hemodialyzed (HD) patients, with prevalence rates between 33% and 82%. Risk factors for chronic pain in HD patients are older age, long-lasting dialysis history, several concomitant diseases, malnutrition, and others. However, chronic pain assessment in HD patients is rarely performed by specialists in pain medicine, with relevant consequences in terms of diagnostic and treatment accuracy.

View Article and Find Full Text PDF

Asthma is a complex disease with varied clinical manifestations resulting from the interaction between environmental and genetic factors. While chronic airway inflammation and hyperresponsiveness are central features, the etiology of asthma is multifaceted, leading to a diversity of phenotypes and endotypes. Although most research into the genetics of asthma focused on the analysis of single nucleotide polymorphisms (SNPs), studies highlight the importance of structural variations, such as copy number variations (CNVs), in the inheritance of complex characteristics, but their role has not yet been fully elucidated in asthma.

View Article and Find Full Text PDF

Genetic landscape in undiagnosed patients with syndromic hearing loss revealed by whole exome sequencing and phenotype similarity search.

Hum Genet

January 2025

Division of Hearing and Balance Research, National Institute of Sensory Organs, NHO Tokyo Medical Center, 2-5-1 Higashigaoka, Meguro-Ku, Tokyo, 152-8902, Japan.

There are hundreds of rare syndromic diseases involving hearing loss, many of which are not targeted for clinical genetic testing. We systematically explored the genetic causes of undiagnosed syndromic hearing loss using a combination of whole exome sequencing (WES) and a phenotype similarity search system called PubCaseFinder. Fifty-five families with syndromic hearing loss of unknown cause were analyzed using WES after prescreening of several deafness genes depending on patient clinical features.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!