Scleroderma

Scleroderma is a chronic autoimmune connective tissue disease characterized by fibrosis of the skin and internal organs. Its pathogenesis involves microvascular damage, immune dysregulation, and fibroblast activation leading to excessive collagen deposition. Clinically, it presents with skin thickening and hardening, often accompanied by visceral involvement such as interstitial lung disease, pulmonary arterial hypertension, scleroderma renal crisis, and gastrointestinal dysfunction. Management relies on immunomodulation, antifibrotic agents, and organ‐specific supportive care, with emphasis on early diagnosis and multidisciplinary collaboration. In biomedical research and data platforms, scleroderma serves as a key model for studying precision phenotyping, biomarker discovery, and targeted therapies.

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16 datasets

Comparing HLA Shared Epitopes in French Caucasian Patients with Scleroderma

Comparing HLA Shared Epitopes in French Caucasian Patients with Scleroderma

免疫遗传学硬皮病分子机制

This dataset focuses on immunogenetic research of scleroderma (SSc), aiming to analyze HLA allele frequencies in French Caucasian patients and evaluate the impact of two common amino acid sequences (67FLEDR71 and 71TRAELDT77) shared by HLA-DRB and DQB susceptibility alleles on disease development. The dataset includes HLA-DRB and DQB typing data from 468 healthy controls and 282 patients with SSc, supporting FLEDR and TRAELDT motif analyses, stratified by clinical subtypes (e.g., diffuse SSc) and autoantibody status (e.g., anti-topoisomerase I antibody ATA). Additionally, standardized HLA-DRβ1 and DRβ5 reverse transcriptase Taqman PCR assays were developed to quantify mRNA expression in 20 subjects with specific HLA haplotypes (HLA-DRB1*15 and/or DRB1*11). Findings indicate that the FLEDR motif is significantly associated with diffuse SSc and the ATA-positive subgroup, with its increase primarily due to higher frequencies of HLA-DRB1*11 and DRB1*15 haplotypes, and the motif is always carried by the most abundantly expressed β chain (β1 or β5). This dataset provides critical data for understanding genetic susceptibility and autoantibody mechanisms in scleroderma, opening new directions for potential therapeutic applications targeting the FLEDR peptide-binding groove.

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Human Urine-derived Stem Cells Ameliorate Bleomycin-induced Skin Fibrosis in a Mouse Scleroderma Model

Human Urine-derived Stem Cells Ameliorate Bleomycin-induced Skin Fibrosis in a Mouse Scleroderma Model

干细胞治疗硬皮病研究

This dataset provides raw data for the manuscript entitled 'Human Urine-derived Stem Cells Ameliorate Bleomycin-induced Skin Fibrosis in a Mouse Scleroderma Model'. It includes experimental data related to the treatment of bleomycin-induced scleroderma mouse models with human urine-derived stem cells (USCs), potentially covering physiological parameters, histological images, molecular biology assays (e.g., gene expression, protein levels), or cellular functional analyses. The dataset supports research on the efficacy and mechanisms of stem cell therapy for skin fibrosis in scleroderma (systemic sclerosis), offering experimental evidence for regenerative medicine and autoimmune disease treatment.

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Sonographic Skin Features and Shear Wave Elastography in Distinguishing Active from Inactive Morphea Lesions: A Case-control Study

Sonographic Skin Features and Shear Wave Elastography in Distinguishing Active from Inactive Morphea Lesions: A Case-control Study

超声成像硬皮病研究

This dataset is a medical imaging research dataset focused on Morphea, a subtype of localized scleroderma. It employs a case-control study design to collect sonographic skin features and shear wave elastography data from both active and inactive Morphea lesions. The dataset includes supplementary figures used to distinguish between the two lesion states, with data derived from clinical ultrasound examinations of patients. It is primarily applied in dermatology and rheumatology, supporting research on imaging-based diagnosis, disease activity assessment, and treatment monitoring for scleroderma.

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SPIN-HAND Feasibility Trial Results

SPIN-HAND Feasibility Trial Results

硬皮病治疗干预

This dataset originates from the feasibility trial of the SPIN-HAND online hand exercise program developed by the Scleroderma Patient-centered Intervention Network (SPIN). It contains quantitative outcome data from the trial, which aimed to evaluate the feasibility of an online self-help hand exercise program designed to improve hand function in patients with scleroderma, providing a basis for future full-scale randomized controlled trials. The data includes clinical assessment metrics from trial participants and is primarily used for research in rheumatology, rehabilitation medicine, intervention effectiveness, and clinical trial methodology.

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