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National Registry for Childhood Onset Scleroderma I: Insights from the first 341 juvenile localized scleroderma patients.
J Scleroderma Relat Disord
September 2024
University of Pittsburgh, Pittsburgh, PA, USA.
Article Synopsis
- The study focuses on juvenile-onset localized scleroderma and aims to raise awareness about its often-overlooked symptoms and the importance of timely treatment.
- The research involved 341 patients, primarily female and Caucasian, showing that many experience additional symptoms outside of skin manifestations, particularly in musculoskeletal and neurological areas.
- Results indicate that early diagnosis and systemic therapy lead to significant improvements in patients' conditions, emphasizing the need for healthcare providers to recognize and treat this disease earlier.
What are Juvenile-onset systemic sclerosis providers thoughts, experiences, and reasons for autologous stem cell transplant? Result of a multinational survey.
J Scleroderma Relat Disord
November 2024
Division of Rheumatology, Department of Pediatrics, School of Medicine, University of Pittsburgh, Pittsburgh, PA, USA.
Article Synopsis
- - The study aimed to assess pediatric specialists' views on using autologous stem cell transplantation (ASCT) for juvenile-onset systemic sclerosis (jSSc), a serious condition with no prior formal research on this topic.
- - An electronic survey was distributed to rheumatology specialists, with 69% responding; all had considered ASCT for jSSc patients, primarily recommending it for rapidly progressive disease and severe symptoms.
- - The findings suggest that while there is awareness and consideration of ASCT among pediatric rheumatologists, there is variability in referral practices, highlighting the need for more research to develop standardized guidelines.
Capillaroscopy in the daily clinic of the pediatric rheumatologist.
Best Pract Res Clin Rheumatol
September 2024
Department of Rheumatology, Ghent University Hospital, Belgium; Faculty of Internal Medicine, Ghent University, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium.
Article Synopsis
- Nailfold capillaroscopy is becoming increasingly important in pediatric rheumatology, aiding in the diagnosis and monitoring of various conditions through a simple, non-invasive procedure.
- The review highlights essential background on capillaroscopy devices, standardized interpretations, and typical variations observed in healthy children versus those with conditions like Raynaud's phenomenon and systemic diseases.
- It also identifies gaps in current research, suggesting a need for further studies on the use of nailfold capillaroscopy in pediatric connective tissue diseases.
Refractory and Recurrent Skin Manifestations in an Adult With Selective Immunoglobulin M Deficiency.
Cureus
April 2024
Department of Dermatology, Graduate School of Medicine, The University of Tokyo, Tokyo, JPN.
Selective immunoglobulin M (IgM) deficiency (sIgMD) is a rare immunodeficiency disorder characterized by decreased serum levels of IgM. Symptoms of sIgMD include repeated infections and allergic manifestations such as asthma and allergic rhinitis. The etiology and pathology of sIgMD remain largely unknown.
View Article and Find Full Text PDFUltrasonographic Evaluation of Juvenile Localized Scleroderma: Enhancing Objectivity in Diagnosis and Management.
Curr Med Imaging
September 2024
Department of Pediatric Rheumatology, University of Health Sciences, Ümraniye Training and Research Hospital, Istanbul, Turkey.
Background: Although clinical assessment has historically been the primary method used for pediatric localized sclerosis (LS) diagnosis and staging, highfrequency ultrasonography (HFUS) is being investigated as a more accurate evaluation method for lesion.
Objectives: This study aimed to assess, compare dermal and subcutaneous tissue characteristics and enhance enhance lesion staging in pediatric LS patients using HFUS.
Methods: Twenty two LS patients were cross-sectionally evaluated with B-mode ultrasonography.
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