Scleroderma is an autoimmune disease characterized by early inflammatory infiltrates followed by fibrosis in the skin and internal organs. CREST is a relatively benign cutaneous variant of scleroderma that features calcinosis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectases. Glomerulonephritis is a rare association of CREST. We are reporting a patient with CREST who developed glomerulonephritis and had anticentromere and antineutrophil cytoplasmic autoantibodies (ANCA) in her serum.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1111/j.1468-3083.2005.01231.x | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Objective: Scleroderma-associated autoantibodies (SSc-Abs) are specific in participants (pts) with systemic sclerosis and are associated with organ involvement. Our objective was to assess the influence of baseline SSc-Abs on the trajectories of the clinical outcome assessments (COAs) in a phase III randomized controlled trial.
Methods: We used data on both the groups who received placebo (Pbo) and tocilizumab from the focuSSced trial.
Differences in the autoantibody phenotypes and long-term outcomes between juvenile- and adult-onset systemic sclerosis.
Mod Rheumatol
January 2025
Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
[Objective] To investigate differences in autoantibodies, clinical features, and long-term outcomes between juvenile- and adult-onset systemic sclerosis (SSc). [Methods] Autoantibodies and survival rates over a maximum of 20 years were retrospectively analyzed in 504 Japanese patients with SSc (juvenile-onset SSc, n=17; adult-onset SSc, n=487) using data from Kyoto University Registry. [Results] The autoantibodies observed were anti-topoisomerase-I (71% vs.
View Article and Find Full Text PDFpDCs, type 1 IFN, and the female predileXion of SSc.
J Exp Med
March 2025
Department of Biomedical Sciences, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Systemic sclerosis (SSc) is a debilitating autoimmune disease that preferentially afflicts women. The molecular origins of this female bias are unclear. A new study of plasmacytoid dendritic cells from SSc patients by Du et al.
View Article and Find Full Text PDFExploring New and Potential Indications for Botulinum Toxin Treatment: An Updated Literature Review.
Cureus
December 2024
General Surgery, Mercy General Hospital, Sacramento, USA.
Botulinum toxin (BoNT) has traditionally been utilized to relieve tension in muscular and connective tissue diseases (CTD). However, its usage has rapidly expanded and now encompasses usage for neurological, gastrointestinal, psychological, cardiovascular, ophthalmology, orthopedics, and more. More recently, its usage has been utilized for sequelae of CTDs such as Raynaud's disease and reduced oral aperture secondary to scleroderma/systemic sclerosis.
View Article and Find Full Text PDFCritical role for Transglutaminase 2 in scleroderma skin fibrosis and in the development of dermal sclerosis in a mouse model of scleroderma.
Arthritis Rheumatol
January 2025
Division of Medicine, Department of Inflammation and Rare Diseases, UCL Centre for Rheumatology, University College London, London, NW3 2PF, UK.
Objective: Scleroderma is a life-threatening autoimmune disease characterized by inflammation, tissue remodelling, and fibrosis. This study aimed to investigate the expression and function of transglutaminase 2 (TGM2) in scleroderma skin and experimentally-induced dermal fibrosis to determine its potential role and therapeutic implications.
Methods: We performed immunohistochemistry on skin sections to assess TGM2 expression and localisation, and protein biochemistry of both SSc-derived and healthy control dermal fibroblasts to assess TGM2 expression, function and ECM deposition in the presence of a TGM2 and TGFβ neutralizing antibodies and a small molecule inhibitor of the TGFβRI kinase.
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!