A esclerodermia sistêmica é uma doença crônica, que talvez seja autoimune (o que significa que o próprio sistema de defesa do corpo ataca seus tecidos por engano). Ela é caracterizada pela produção excessiva de colágeno, uma proteína que causa o endurecimento e espessamento dos tecidos do corpo. Existem dois tipos principais de esclerodermia sistêmica: a cutânea limitada e a cutânea difusa. A classificação é feita de acordo com a extensão da pele afetada. Há uma relação entre a quantidade de pele atingida e o grau de envolvimento dos órgãos e sistemas internos do corpo. A esclerodermia sistêmica pode se manifestar de várias formas, incluindo: fibrose pulmonar (endurecimento dos pulmões), Síndrome de Raynaud (um problema de circulação que afeta principalmente os dedos das mãos e dos pés), telangiectasias no sistema digestório (pequenos vasos sanguíneos dilatados no aparelho digestivo), pressão alta nos rins e/ou pressão alta nas artérias dos pulmões.
Introdução
O que você precisa saber de cara
A esclerodermia sistêmica é uma doença crônica, que talvez seja autoimune (o que significa que o próprio sistema de defesa do corpo ataca seus tecidos por engano). Ela é caracterizada pela produção excessiva de colágeno, uma proteína que causa o endurecimento e espessamento dos tecidos do corpo. Existem dois tipos principais de esclerodermia sistêmica: a cutânea limitada e a cutânea difusa. A classificação é feita de acordo com a extensão da pele afetada. Há uma relação entre a quantidade de pele atingida e o grau de envolvimento dos órgãos e sistemas internos do corpo. A esclerodermia sistêmica pode se manifestar de várias formas, incluindo: fibrose pulmonar (endurecimento dos pulmões), Síndrome de Raynaud (um problema de circulação que afeta principalmente os dedos das mãos e dos pés), telangiectasias no sistema digestório (pequenos vasos sanguíneos dilatados no aparelho digestivo), pressão alta nos rins e/ou pressão alta nas artérias dos pulmões.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 49 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 112 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
6 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial, Not applicable.
Major connective tissue mitoattractant secreted by vascular endothelial cells. Promotes proliferation and differentiation of chondrocytes. Is involved in the stimulation of osteoblast differentiation and has a critical role in osteogenesis (PubMed:39414788). Mediates heparin- and divalent cation-dependent cell adhesion in many cell types including fibroblasts, myofibroblasts, endothelial and epithelial cells. Enhances fibroblast growth factor-induced DNA synthesis
Secreted, extracellular space, extracellular matrixSecreted
Kyphomelic dysplasia
An autosomal recessive skeletal dysplasia characterized by bowing of the limbs primarily affecting the femora, along with short stature, short and wide iliac wings, horizontal acetabular roof, platyspondyly, metaphyseal flaring and distinctive facial features that include prominent forehead, micrognathia, microstomia, cleft palate and low set ears.
Transcription factor that plays a critical role in innate immunity by activating expression of type I interferon (IFN) IFNA and INFB and inflammatory cytokines downstream of endolysosomal toll-like receptors TLR7, TLR8 and TLR9 (PubMed:11303025, PubMed:15695821, PubMed:22412986, PubMed:25326418, PubMed:32433612). Regulates the transcription of type I IFN genes (IFN-alpha and IFN-beta) and IFN-stimulated genes (ISG) by binding to an interferon-stimulated response element (ISRE) in their promoters
CytoplasmNucleus
Inflammatory bowel disease 14
A chronic, relapsing inflammation of the gastrointestinal tract with a complex etiology. It is subdivided into Crohn disease and ulcerative colitis phenotypes. Crohn disease may affect any part of the gastrointestinal tract from the mouth to the anus, but most frequently it involves the terminal ileum and colon. Bowel inflammation is transmural and discontinuous; it may contain granulomas or be associated with intestinal or perianal fistulas. In contrast, in ulcerative colitis, the inflammation is continuous and limited to rectal and colonic mucosal layers; fistulas and granulomas are not observed. Both diseases include extraintestinal inflammation of the skin, eyes, or joints.
Possible role in axon guidance through interaction with RTN4R (Microbial infection) Acts as a receptor for adeno-associated virus and is involved in adeno-associated virus infection through endocytosis system
Cytoplasmic granule membraneGolgi apparatus membraneGolgi apparatus, trans-Golgi network membraneCell membrane
Receptor for CCL19 and chemerin/RARRES2. Does not appear to be a signaling receptor, but may have a role in modulating chemokine-triggered immune responses by capturing and internalizing CCL19 or by presenting RARRES2 ligand to CMKLR1, a functional signaling receptors. Plays a critical role for the development of Th2 responses
Cell membrane
May act as a scaffolding protein within caveolar membranes (PubMed:11751885). Forms a stable heterooligomeric complex with CAV2 that targets to lipid rafts and drives caveolae formation. Mediates the recruitment of CAVIN proteins (CAVIN1/2/3/4) to the caveolae (PubMed:19262564). Interacts directly with G-protein alpha subunits and can functionally regulate their activity (By similarity). Involved in the costimulatory signal essential for T-cell receptor (TCR)-mediated T-cell activation. Its bind
Golgi apparatus membraneCell membraneMembrane, caveolaMembrane raftGolgi apparatus, trans-Golgi networkCytoplasm
Lipodystrophy, congenital generalized, 3
A form of congenital generalized lipodystrophy, a metabolic disorder characterized by a near complete absence of adipose tissue, extreme insulin resistance, hypertriglyceridemia, hepatic steatosis and diabetes mellitus. CGL3 inheritance is autosomal recessive.
A beta chain of antigen-presenting major histocompatibility complex class II (MHCII) molecule. In complex with the alpha chain HLA-DRA, displays antigenic peptides on professional antigen presenting cells (APCs) for recognition by alpha-beta T cell receptor (TCR) on HLA-DRB1-restricted CD4-positive T cells. This guides antigen-specific T-helper effector functions, both antibody-mediated immune response and macrophage activation, to ultimately eliminate the infectious agents and transformed cells
Cell membraneEndoplasmic reticulum membraneLysosome membraneLate endosome membraneAutolysosome membrane
Medicamentos aprovados (FDA)
2 medicamentos encontrados nos registros da FDA americana.
Variantes genéticas (ClinVar)
57 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
39 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 11.188
Autologous stem cell transplant for severe, progressive juvenile systemic sclerosis.
Juvenile systemic sclerosis (jSSc) is a rare, chronic, autoimmune disease in children/adolescents and is associated with significant morbidity, skin thickening/hardening (scleroderma), organ toxicity and sub-optimal therapeutic options. In this report, autologous stem cell transplantation is associated with clinical improvement in a 17-year-old with refractory jSSc.
Automatic assessment of lung involvement in systemic sclerosis using deep learning.
Systemic sclerosis (SSc) is a relatively uncommon connective tissue disorder, commonly manifesting as interstitial lung disease (ILD) and affecting both the lung parenchyma and the modification of the space between endothelium and epithelium. Imaging modalities like computed tomography (CT) scans are essential for diagnosing and revealing specific abnormal findings (ILD patterns) in SSc, such as reticulation and Ground-glass opacity (GGO). To enhance diagnostic precision and minimize human error, we leverage deep learning (DL) techniques. In our study, we collected and annotated a new public dataset from 22 individuals, encompassing 2190 lung CT scan slices. After preprocessing and exclusion of slices without abnormalities, 1777 slices from 17 patients were used for model training and validation, and 413 slices from five patients were reserved for independent testing. We use a specialized U-net model to segment these patterns, categorizing them into reticulation or GGO, and employ an automated algorithm to outline lung areas in each CT slice. The model's objective is to quantify the patient's lung involvement in SSc by calculating the total identified GGO and reticulation areas across all slices and normalizing this by the total lung surface area. The U-net model shows promising results in segmenting both reticulation and a combination of GGO and reticulation, as indicated by Dice coefficients of 87.22% and 86.20%, respectively. Furthermore, the automated algorithm effectively outlines the lung region in each slice, enabling accurate measurement of lung involvement in SSc patients. In conclusion, using DL using the U-Net model and an automated algorithm has shown promising results in accurately segmenting and quantifying lung involvement in Scleroderma patients using CT scans.
When atrioventricular block paves the way to a more severe diagnosis: a case report.
Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by widespread fibrosis of skin and internal organs. Cardiac involvement is common but often subclinical, and advanced conduction abnormalities such as complete atrioventricular block are rarely the initial manifestation. A 42-year-old man presented with fatigue and episodes of syncope. Electrocardiography confirmed complete AV block. Physical examination revealed diffuse skin tightening and Raynaud's phenomenon. Immunological testing detected high-titre antinuclear antibodies and anti-topoisomerase I, confirming diffuse cutaneous SSc. Further evaluation disclosed multiorgan involvement: high-resolution CT showed pulmonary fibrosis and right heart catheterization confirmed severe precapillary pulmonary hypertension. The patient underwent permanent dual-chamber pacemaker implantation and was started on immunosuppressive therapy and pulmonary vasodilators. On follow-up, his bradycardia resolved and exercise tolerance improved, indicating partial clinical improvement. This case highlights an atypical cardiac onset of diffuse SSc with high-grade conduction disease. It underscores the importance of considering systemic autoimmune disorders in patients with unexplained high-degree AV block, especially in the presence of characteristic skin findings. Comprehensive assessment revealed concurrent pulmonary and gastrointestinal involvement, reflecting the multisystem nature of SSc. Early recognition allowed a coordinated multidisciplinary treatment strategy, including device implantation and disease-modifying therapy, which ultimately stabilized the patient's condition. This case emphasizes the need for early diagnosis and integrated management of cardiac, pulmonary, and gastrointestinal complications in SSc. Although SSc is generally progressive, prompt recognition and treatment of organ involvement may significantly improve patient outcomes and quality of life.
Mechanical gating of tendon fibrogenic transcription in systemic sclerosis.
Fibrosis arises from aberrant tissue repair in systemic sclerosis and is characterized by progressive stiffening across multiple tissues, including tendons. How altered tissue mechanics sustain fibrotic remodeling remains poorly understood, in part because of limited experimental models that capture key biophysical features of fibrosis. Here we develop a modular cantilever-based mechano-culture platform that enables tendon-like constructs to be maintained under controlled static tension. We show that elevated matrix tension induces fibroblast-to-myofibroblast activation and scar-like phenotypes in vitro. Analysis of preclinical and clinical models of systemic sclerosis reveals that increased three-dimensional matrix stiffness inversely correlates with transcription of major profibrotic collagens, while positively regulating genes associated with stromal-immune interactions. Co-culture with bone marrow-derived macrophages overrides tension-dependent suppression of matrix gene expression, suggesting that immune cues can supersede mechanical checkpoints. These findings demonstrate how tissue mechanics orchestrates reciprocal interactions between stromal and immune compartments to drive progressive fibrosis, and establish a reductionist platform for dissecting mechano-regulatory pathways in fibrotic diseases.
Resting Oxygen Consumption Estimates in Scleroderma Can Lead to Underestimation of Cardiac Output.
Accurate resting oxygen consumption (rVO2) quantification is critical for Fick-derived cardiac output calculations. Yet, clinical practice predominantly uses empirical estimations, which can be inaccurate. We evaluated the established rVO₂ prediction equations against direct metabolic cart measurements in systemic sclerosis patients, revealing significant discordances with potential diagnostic implications.
Publicações recentes
Tocilizumab as a Potential Alternative Therapy for Acute Exacerbation of Systemic Sclerosis-Associated Interstitial Lung Disease in a Dialysis Patient Following Immunodeficiency-Associated Lymphoproliferative Disorders.
Screening, monitoring and treatment of interstitial lung disease in systemic sclerosis.
Physical rehabilitation interventions for hand function in people with systemic sclerosis.
Clinical relevance of specific and non-specific autoantibodies in systemic sclerosis without overlap features.
The Effect of Adding Paraffin Bath to the Therapeutic Hand Exercise Programme on Hand Functions in Patients with Systemic Sclerosis: A Randomized, Controlled, Single-Blind Study.
📚 EuropePMC14.729 artigos no totalmostrando 195
Defining quality standards of care in CTD-PAH and management best practices: a Delphi panel consensus.
Rheumatology advances in practiceDiffusing capacity of the lung for carbon monoxide, transfer coefficient of the lung for carbon monoxide and forced vital capacity/diffusing capacity of the lung for carbon monoxide in suspected systemic sclerosis-associated pulmonary hypertension: insights from the ASPIRE registry.
ERJ open researchNeurofascialvascular Training in Systemic Sclerosis: Clinical Outcomes, Proposed Rationale and Home-Program Feasibility. A Case Report.
Modern rheumatology case reportsAutologous stem cell transplant for severe, progressive juvenile systemic sclerosis.
Stem cells translational medicineHuman blood vessel organoids recapitulate key mechanisms of transition from vasculopathy to fibrosis in systemic sclerosis.
Annals of the rheumatic diseasesDefinition, diagnosis, treatment for scleroderma primary heart involvement.
Best practice & research. Clinical rheumatologyAutomatic assessment of lung involvement in systemic sclerosis using deep learning.
Journal of research in medical sciences : the official journal of Isfahan University of Medical SciencesWhen atrioventricular block paves the way to a more severe diagnosis: a case report.
European heart journal. Case reportsPrevalence of comorbidities and medication use in progressive systemic sclerosis in northeast Mexico.
Reumatologia clinicaDigital Ischemia Following Radial Access Unmasking Systemic Sclerosis: Characteristic Intravascular Ultrasound Findings.
JACC. Case reportsCellular therapies for rheumatic disease.
Current opinion in rheumatologyCharacterizing the effects of genetic liability to autoimmune conditions on pregnancy outcomes using Mendelian randomization.
BMC medicineThe immune landscape of systemic sclerosis: from pathogenic mechanisms to precision therapeutic breakthroughs.
Frontiers in immunologyRisk score for early mortality to stratify for intensive SSc therapy in the EUSTAR network: the RESIST score.
Annals of the rheumatic diseasesCentral memory T cells: stem-like biology, pathogenic roles in autoimmunity, and therapeutic applications.
Clinical and experimental rheumatologyPerformance of Circulating Inflammatory Biomarkers and Krebs von den Lungen 6 for Predicting Progressive Pulmonary Fibrosis in Patients With Systemic Sclerosis-Associated Interstitial Lung Disease.
ACR open rheumatologyNanotechnology-Enhanced Photosensitizers: Review of Multifunctional Theranostics for Precision Medicine.
Current pharmaceutical designFibrosis-memory is mediated by IL-3-producing T cells and drives progression of fibrosis.
The Journal of clinical investigationDiffuse Cutaneous Systemic Sclerosis Complicated by Serum Amyloid A Protein Cardiac Amyloidosis and Cardiogenic Shock.
CJC openTransdermal Delivery of Hyaluronate-Conjugated Formyl Peptide Receptor 2 Agonistic Peptide Ameliorates Bleomycin-Induced Skin Fibrosis.
Biomaterials researchOptical coherence tomography angiography: a window on systemic sclerosis microangiopathy.
European journal of internal medicineReal-World Adherence to Systemic Sclerosis Quality Indicators: A Single-Center Quality of Care Study.
The Journal of rheumatologyScleroderma sine-Raynaud in children: the Fibrotic Subtype.
The Journal of rheumatologyIncreased incidence of sudden cardiac death in systemic sclerosis: data from a nationwide cohort study.
Seminars in arthritis and rheumatismStress Echocardiography in the Diagnosis and Evaluation of Pulmonary Hypertension: Practical Recommendations, Haemodynamic Phenotyping, and Application in Adults and Children.
Diagnostics (Basel, Switzerland)Exploring Disease-Specific Risk Factors for Vertebral Fractures in Systemic Sclerosis: Insights from the ScleroRER Study Group.
Journal of clinical medicineLingual Raynaud's Phenomenon: A Case Series and Literature Review.
Journal of clinical medicineICOS+ T helper 2 cells promote the pulmonary fibrosis in systemic sclerosis.
Molecular medicine (Cambridge, Mass.)Mechanical gating of tendon fibrogenic transcription in systemic sclerosis.
Nature communicationsNailfold capillaroscopy and organ involvement in systemic sclerosis: a systematic review.
Best practice & research. Clinical rheumatologyImmune rehabilitation after renal transplantation in autoimmune diseases: Balancing immunosuppression and risk of complications.
Autoimmunity reviewsHematopoietic Stem Cell Transplantation in Rheumatic Diseases.
International journal of rheumatic diseasesSIX1 and PAI-1 emerge as therapeutic targets in systemic sclerosis.
Nature reviews. RheumatologyQuantitative comparative analysis of different CT modalities in systemic sclerosis-associated interstitial lung disease: correlations with lung function, serological biomarkers, and disease stratification.
Journal of thoracic diseaseResting Oxygen Consumption Estimates in Scleroderma Can Lead to Underestimation of Cardiac Output.
Pulmonary circulationAnalysis of the impact of pharmacological treatment on arterial stiffness in patients with connective tissue diseases.
American heart journal plus : cardiology research and practiceHuman MAGI1 expression in endothelial cells protects from the development of localized and systemic scleroderma in mice.
Arthritis research & therapyObjective gastrointestinal involvement in patients with systemic sclerosis: Prevalence and clinico-immunological phenotypes in the REMSCLE registry.
Medicina clinicaDermatoscopy limitation and the critical role of capillaroscopy in the evaluation of systemic sclerosis and Raynaud's phenomenon among African American Veterans.
Seminars in arthritis and rheumatismPrevention and management of gastrointestinal side effects in patients with systemic sclerosis-interstitial lung disease receiving anti-fibrotic therapy: a modified Delphi consensus study.
Clinical and experimental rheumatologyCervical spinal calcinosis in systemic sclerosis presenting with foraminal stenosis, radiculopathy and vertebral artery displacement.
Clinical and experimental rheumatologyAnti-PM/Scl Antibody-Positive Dermatomyositis With Rapidly Progressive Interstitial Lung Disease in a 19-Year-Old Male: Clinical Implications of a Presumptive Diagnosis Based on Line Immunoassay Positivity.
CureusEfficacy and Safety of Tofacitinib Compared to Cyclophosphamide in Early Diffuse Cutaneous Systemic Sclerosis: A Randomized Controlled Trial.
CureusCommunity-based management of gastrointestinal symptoms at end-of-life in scleroderma using subcutaneous medication: a case report.
Annals of palliative medicineRecognizing autoimmunity in a breast cancer survivor with cutaneous fibrosis.
JAAD case reportsNintedanib Attenuates Profibrotic Gene Expression in a 3D Organotypic Culture Model of Systemic Sclerosis Skin Fibrosis.
The Journal of investigative dermatologyScleromyositis - The new frontier.
Best practice & research. Clinical rheumatologyCAR-T therapy for autoimmune rheumatic diseases: navigating clinical frontiers between breakthroughs and uncertainties.
Clinical and experimental medicineLongitudinal Assessment of the Juvenile Systemic Sclerosis Severity Score: Application in the National Registry for Childhood Onset Scleroderma.
ACR open rheumatologySafety and efficacy of blinatumomab in the treatment of refractory systemic sclerosis: a case series.
Annals of the rheumatic diseasesThe "Inflammatory-Mutagenic" Landscape of Systemic Sclerosis (SSc)-Associated Lung Cancer: Distinct Genomics and Therapeutic Implications.
Journal of thoracic oncology : official publication of the International Association for the Study of Lung CancerA retrospective analysis of clinical characteristics of systemic sclerosis-associated interstitial lung disease.
MedicineGlobal trends in systemic sclerosis-related mortality, 2001-2023: an epidemiological analysis using World Health Organization mortality data.
Clinical rheumatologyPrevalence, clinical features, and laboratory predictors of autoimmune hepatitis in systemic sclerosis: A retrospective single-center cohort study.
Clinical rheumatologyCase of generalized morphea complicated with systemic sclerosis.
Dermatology reportsExploring the anti-fibrotic effects of safflower in systemic sclerosis based on metabolomics and gut microbiota analyses.
Frontiers in pharmacologySeptic Pulmonary Embolism in Systemic Sclerosis-Associated Intestinal Pseudo-Obstruction: A Case Report.
Modern rheumatology case reportsAutoimmune and malignant diseases secondary to autoimmune/inflammatory syndrome induced by modeling substances (ASIA-MS): A short communication of a clinical case series.
Reumatologia clinicaIdentification of key macrophage-related genes in systemic sclerosis-associated interstitial lung disease based on single-cell and bulk transcriptomic data.
PloS oneCombination therapy with mycophenolate mofetil, nintedanib, and tocilizumab in a patient with progressive systemic sclerosis-associated interstitial lung disease : A case report and a literature review.
Zeitschrift fur RheumatologieRare ANA patterns and their clinical correlates: a retrospective large-cohort study.
Clinical chemistry and laboratory medicineThe clinical phenotype of anti-Th/To+ patients in systemic sclerosis: a case-control study within the European Scleroderma Trials and Research cohort.
Therapeutic advances in musculoskeletal diseaseSoluble immune checkpoints reflect immune activation and treatment response in high-risk systemic sclerosis patients treated with plasma exchange.
Journal of translational autoimmunityComplement protein C1q induces endothelial apoptosis through C1qBP regulation of mitochondrial function in lung endothelial cells in systemic sclerosis.
Molecular medicine (Cambridge, Mass.)Recent advances in the heart and soul of primary cardiac involvement and cardiovascular disease in systemic sclerosis.
Best practice & research. Clinical rheumatologyCAR-T cell therapy for treatment-refractory rheumatic autoimmune diseases: a systematic review of clinical outcomes and safety profiles.
RMD openBiologic Rescue With Tocilizumab: A Case of Refractory Chronic Pericardial Effusion in Systemic Sclerosis.
JACC. Case reportsNovel cyp51A mutation profile in Aspergillus fumigatus from a patient with systemic lupus erythematosus and limited systemic sclerosis: first clinical case of acquired azole-resistance in Colombia.
BMC infectious diseasesOsteolysis After Interphalangeal Joint Arthrodesis in Systemic Sclerosis: A Case Series.
The Journal of rheumatologyThe multifaceted role of platelets in systemic sclerosis: beyond haemostasis!
Current opinion in hematologyEvaluation of non-invasive techniques for the diagnosis of pulmonary complications in systemic sclerosis and scleroderma-like overlap syndromes: role of lung ultrasound.
Journal of autoimmunityOutcomes Following Surgical Management of Advanced Oral Cavity Cancers in Patients with Scleroderma: A Case Series.
The Annals of otology, rhinology, and laryngologyReciprocal regulation of fibroblast-macrophage equilibrium governs skin integrity.
Nature immunologyRaynaud's phenomenon and digital ulceration in systemic sclerosis.
Best practice & research. Clinical rheumatologyFaecal incontinence in systemic sclerosis: A narrative review.
La Revue de medecine interneAnti-CD146 Autoantibodies: The First Biologic Markers Associated With Occupational Exposure in Systemic Sclerosis.
ACR open rheumatologyScleroderma clinical trials consortium classification criteria for systemic sclerosis heart involvement.
Rheumatology (Oxford, England)Nintedanib in systemic sclerosis-associated interstitial lung disease: real-world cohort study on tolerability and discontinuation.
Clinical and experimental rheumatologyDistinct by Design: Unraveling the Unique Clinical and Transcriptomic Identity of Juvenile Scleromyositis Overlap Compared to Juvenile Systemic Sclerosis and Juvenile Dermatomyositis: Implications for Care and Pathogenesis.
ACR open rheumatologySpatial transcriptomics reveals mechanism of autoimmunity driven by internalized autoantibodies.
medRxiv : the preprint server for health sciencesA university career in basic and applied avian immunology: important contributions of chicken models for autoimmune diseases.
Frontiers in physiologyComputed tomography of interstitial lung disease in systemic sclerosis: dataset and deep learning model for pulmonary lesion segmentation.
ReumatismoSystematic Review and Model-Based Meta-Analysis of Targeted Drugs for Systemic Sclerosis.
PharmaceuticsIncreased Mortality Among Young Systemic Sclerosis Patients During the COVID-19 Pandemic: A Nationwide Data Analysis from Thailand.
Life (Basel, Switzerland)Associations Between Depression and Reduced Quality of Life in Women with Non-Radiographic Axial Spondyloarthritis: A Cross-Sectional Study.
BiomedicinesEpigenetic Therapies for Inflammatory and Immune-Mediated Skin Diseases.
BiomedicinesTemporomandibular joint disorders in patients with systemic sclerosis: a cross-sectional study.
BMC rheumatologyStem Cell-Based Therapies in Autoimmune Diseases: Current Evidence, Unmet Needs, and Future Directions-A Closing Editorial Review.
CellsThe rheumatry registry: design and data collection methods.
BMC rheumatologyMetabolic masqueraders of paediatric and adult rheumatic diseases.
Nature reviews. RheumatologyPoints to consider for reporting digital ulcers in systemic sclerosis interventional studies: An initiative from the world Scleroderma Foundation digital ulcer ad hoc committee.
Autoimmunity reviewsWNT5A induces profibrotic activation of human macrophages via pSTAT3.
Rheumatology (Oxford, England)Extended temporomandibular joint prosthetic reconstruction of total condylysis in the setting of severe scleroderma: a case report.
Oral and maxillofacial surgeryCrowned Dens Syndrome Mimicking Atlantoaxial Infection in a Patient With Systemic Sclerosis: A Case Report.
Case reports in rheumatologyCoping and Resilience in Chinese Couples with Connective Tissue Diseases: A Culturally Informed Qualitative Explanation.
Patient preference and adherenceA randomised open label pilot trial comparing mycophenolate mofetil with no immunosuppression in limited cutaneous systemic sclerosis (MINIMISE-Pilot).
Rheumatology (Oxford, England)Pericardial Decompression Syndrome After Large-Volume Pericardiocentesis in Systemic Sclerosis.
JACC. Case reportsImprovements in Outcomes for Systemic Sclerosis-Associated Interstitial Lung Disease With Expanded Treatment Options.
Arthritis & rheumatology (Hoboken, N.J.)Integrating B Cell Differentiation Model With Real-World Data Informs Determinants for Antibody-Secreting Cell Depletions in Systemic Sclerosis.
CPT: pharmacometrics & systems pharmacologyHyaluronic acid fillers in systemic sclerosis and localized scleroderma: A systematic review.
Autoimmunity reviewsProton Pump Inhibitor Use and Treatment Satisfaction in Spanish Patients with Systemic Sclerosis: Insights from a Nationwide Survey.
Gastroenterologia y hepatologiaLong Non-Coding RNA H19 mediates STAT3 dependent activation of keratinocytes and fibroblasts in Systemic Sclerosis skin.
Arthritis & rheumatology (Hoboken, N.J.)Evaluating context effects on PHQ-8 somatic item scores among people with a chronic medical condition: a scleroderma patient-centred intervention network randomised experiment.
Epidemiology and psychiatric sciencesPulmonary Venous Vasculopathy in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension With Reduced Diffusion Capacity for Carbon Monoxide.
Pulmonary circulationPlasma GDF-15 and PSP-D Predict the Development of Pulmonary Arterial Hypertension in Systemic Sclerosis.
Pulmonary circulationReduced retinal microvascular density in patients with mixed connective tissue disease: an exploratory pilot study on the interplay between aging, renal function, and complement system.
Frontiers in immunologyCase Report: Unusual persistent elevation of troponin I-systemic sclerosis masked by acute myocardial infarction.
Frontiers in immunologyMesial temporal lobe sclerosis and systemic sclerosis in a young female with cognitive and psychiatric manifestations.
Industrial psychiatry journalCarbon dioxide vs. warm-water hand baths in systemic sclerosis with secondary Raynaud's syndrome - A capillaroscopy centered randomized controlled trial.
Microvascular researchIncidence, prevalence and clinical features of systemic sclerosis patients from a southern Brazilian city.
Advances in rheumatology (London, England)Drug development in systemic sclerosis: novel therapeutics to watch.
Expert review of clinical immunologySafety and Tolerability of Nintedanib in Japanese Patients with Progressive Fibrosing Interstitial Lung Diseases: Final Results of 2-Year Post-Marketing Surveillance.
Advances in therapyA systematic review of therapeutic procedures for the treatment of morphea and systemic sclerosis.
Lasers in medical scienceInflammatory coronary arteritis complicated by myocardial infarction: an exceptional manifestation of systemic sclerosis.
Oxford medical case reportsPrevalence and clinical relevance of systematically tested antimitochondrial antibodies in systemic sclerosis.
Arthritis research & therapyBispecific T cell engagers for treatment-refractory autoimmune connective tissue diseases.
Nature medicineAdipocyte-Specific Deletion of Sine Oculis Homeobox Homolog 1 Inhibits Lipolysis and Reduces Skin Fibrosis.
JCI insightSolar purpura preceding Raynaud's phenomenon in limited systemic sclerosis.
Internal and emergency medicineCytometry-based blood immune cell markers associated with clinical outcomes in systemic sclerosis: protocol for a systematic review.
BMJ openManagement of pulmonary arterial hypertension in systemic sclerosis: from classical treatments to new horizons.
European respiratory review : an official journal of the European Respiratory SocietyElevated lipid peroxidation biomarkers in autoimmune diseases: A systematic review and meta-analysis.
Autoimmunity reviewsAssessing the structural validity of the SCL-Dep6 depression subscale using rasch analysis.
Journal of affective disordersChaperone-mediated autophagy supports organ regeneration and fibroblast quiescence in mouse models of fibrosis.
Science translational medicineIntercellular communication landscape and its working mechanism in systemic sclerosis-associated interstitial lung disease.
Rheumatology (Oxford, England)Systemic sclerosis-associated pulmonary arterial hypertension and pulmonary fibrosis: exploring biomarker discriminators with advanced omics in a Caucasian cohort.
Frontiers in immunologyAn Under-Recognized Manifestation of Systemic Sclerosis: Macrovascular Peripheral Arterial Disease.
CureusNovel Compounds as LPAR1 Inhibitors for Treating Systemic Sclerosis.
ACS medicinal chemistry lettersA potent inhibitor of PAI-1, MDI-2517, mitigates disease severity in a preclinical systemic sclerosis model.
JCI insightA CD57+ CD8 T cell subset links cytotoxic T cell cytotoxicity to fibrotic lung disease in systemic sclerosis.
The Journal of clinical investigationMicrovascular markers in systemic sclerosis: a comparative OCT-angiography and nailfold videocapillaroscopy study.
Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle OphthalmologieAn autopsy-proven case of normotensive scleroderma renal crisis with refractory pleuroperitoneal effusion.
CEN case reportsOral and maxillofacial manifestations of systemic sclerosis.
Best practice & research. Clinical rheumatologyUnderrecognition of Fecal Incontinence in Systemic Sclerosis: Discrepancies Between the Rome IV Diagnostic Questionnaires and the UCLA Scleroderma Clinical Trials Consortium Gastrointestinal 2.0 Questionnaire.
The Journal of rheumatologyWhen Infection Leaves Its Mark: Calcinosis in Systemic Sclerosis.
The Journal of rheumatologyColonic Telangiectasias Associated With Systemic Sclerosis.
The Journal of rheumatologyEffects of home-based inspiratory muscle training on sympatho-vagal balance, quality of life, and inflammatory profile in patients with systemic sclerosis-associated interstitial lung disease.
European journal of medical researchSecond to Fourth Digit Ratio (2D:4D) in Female Patients With Systemic Sclerosis: Evidence for Prenatal Androgen Exposure.
American journal of human biology : the official journal of the Human Biology CouncilAssessing digital vasculopathy in systemic sclerosis.
Rheumatology (Oxford, England)Advancing drug development for systemic sclerosis by prioritizing findings from human genetic association studies.
Rheumatology (Oxford, England)Exploring the optimal follow-up for systemic sclerosis patients: study protocol for a Dutch multicenter randomized controlled trial.
TrialsProbabilistic Clustering Using Multivariate Growth Mixture Model in Clinical Settings-A Scleroderma Example.
Statistics in medicineGastric disease in systemic sclerosis: Spectrum, challenges, and insights from a systematic literature review.
Seminars in arthritis and rheumatismSuccessful use of upadacitinib for the improvement of vitiligo-like and perifollicular dyspigmentation (salt-and-pepper skin) in systemic sclerosis.
Dermatology reportsPrimary Biliary Cholangitis Pathogenesis: A Pathophysiology-Based Narrative Review.
International journal of molecular sciencesAnifrolumab-A Potential New Systemic Sclerosis Treatment.
Journal of clinical medicineCalcinosis Cutis Universalis: A Review of Therapeutic Strategies and Surgical Management.
Journal of clinical medicinePrevalence of effective contraceptive use among patients with rheumatic diseases: a descriptive study.
BMC rheumatologyEfficacy of rituximab treatment in a post-COVID-19 vaccine myositis overlapping to systemic sclerosis: a histological follow-up.
Clinical and experimental rheumatologyAssociation between autoantibodies and interstitial lung disease in autoimmune disease patients: a retrospective study.
Clinical and experimental rheumatologyDiagnostic model and identification of IGFBP2 as an aging-related biomarker and therapeutic target for systemic sclerosis-associated interstitial lung disease through integrated bioinformatics and machine learning approaches.
International journal of surgery (London, England)Genetic and Genomic Analysis of Systemic Sclerosis: A Narrative Review of the Recent Advancement.
JMA journalCan Baseline Features Predict Progression to Defined CTD? Insights from a Minimum 5-Year Follow-Up Study of 504 Patients with UCTD.
Modern rheumatologyInterstitial lung disease in systemic autoimmune rheumatic diseases: radiologic and histologic correlations.
Rheumatology advances in practiceEvidence For a Fibrogenic Interaction Between the Aryl Hydrocarbon Receptor and the Wnt/β-Catenin Pathways in Human Keratinocytes and Fibroblasts.
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacologyQuantifying the Prevalence of Disorders of Gut Brain Interaction in Systemic Sclerosis.
Neurogastroenterology and motilityEngaging the PD-1 pathway in systemic sclerosis attenuates inflammation-driven fibrosis.
Annals of the rheumatic diseasesEvaluation of serial cardiovascular magnetic resonance monitoring and immunosuppressive therapy in predicting outcomes in systemic sclerosis.
RMD openAnalysis of the Dynamics of Clinical Parameters and Serum Angiogenic Factors in Systemic Sclerosis Patients Undergoing Tocilizumab Treatment.
Experimental dermatologyClinical Image: Esophageal dilation and stenosis in systemic sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)Anti-CENP-B polarity divides SLE: divergent clinical-immune phenotypes and distinct treatment responses.
Frontiers in immunologyAge and onset timing of Raynaud's phenomenon and first non-Raynaud symptom as prognostic factors in systemic sclerosis: a retrospective analysis from the Italian national multicenter Systemic Sclerosis Progression INvestiGation registry of the Italian Society for Rheumatology (SPRING-SIR).
Therapeutic advances in musculoskeletal diseaseCystathionine γ-Lyase-Dependent S-Sulfhydration of Smad3: A Novel Target to Alleviate Fibrosis in Systemic Sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)Clinical Images: Complete Distal Phalanx Resorption in Systemic Sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)The impact of vertebral fractures on pulmonary function tests in patients with interstitial lung disease: a cross-sectional study.
Respiratory researchReactive oxygen species driven immune inflammation in systemic sclerosis: mechanisms and emerging therapeutics.
Drug discovery todayDelayed gastric emptying identifies a high-risk clinical subgroup in patients with systemic sclerosis.
Rheumatology (Oxford, England)Suppression of interferon signaling via small-molecule modulation of TFAM.
eLifeCleavage and Polyadenylation Specificity Factor Subunit 5 Regulates Pulmonary Artery Smooth Muscle Expansion and Hypoxic Response.
MedCommReproductive Outcomes and Fertility Patterns in Women with Systemic Sclerosis: A Multicentre Observational Study.
Mediterranean journal of rheumatologyEarly detection and risk stratification in autoimmune-related interstitial lung disease: a state-of-the-art review.
Respiratory researchAnti-CD19 CAR T cells for pediatric patients with treatment-refractory autoimmune diseases.
Nature medicineGain of function NOTCH4 variants disrupt angiogenesis in systemic sclerosis.
Annals of the rheumatic diseasesResearch on the mechanism of Carthamus tinctorius L. against skin fibrosis in systemic sclerosis based on serum pharmacochemistry, network pharmacology, and experimental validation.
Journal of ethnopharmacologyFlare incidences of pre-existing rheumatologic diseases in patients with solid tumors receiving immune checkpoint inhibitors: A systematic review and meta-analysis.
Seminars in arthritis and rheumatismThe Spectrum of Uveitis and Retinal Vasculitis in Systemic Sclerosis: A Case Series.
Ocular immunology and inflammationImpact of Hydroxychloroquine on Mortality and Cardiovascular Outcomes in Systemic Sclerosis: A Retrospective Cohort Study.
International journal of rheumatic diseasesCardiac involvement in systemic sclerosis: mechanisms, manifestations and management.
Rheumatology (Oxford, England)Juvenile systemic sclerosis.
Best practice & research. Clinical rheumatologyEvaluation of corneal and lens properties in patients with systemic sclerosis: a densitometric analysis.
Clinical & experimental optometryTPI1 and TPM4 are strong candidate RNA biomarkers for systemic sclerosis.
Arthritis research & therapySpectroscopic and machine learning approaches for clinical subtyping in systemic sclerosis.
Scientific reports[Filgotinib treatment of calcinosis in centromere-antibody-positive systemic sclerosis].
Zeitschrift fur RheumatologieExploratory evaluation of early 68Ga-FAPI-46 PET/CT volume-based parameters in systemic sclerosis-associated interstitial lung disease.
European journal of nuclear medicine and molecular imagingCytokine Storm Following Lung Transplant in the Setting of Alistipes finegoldii Bacteremia: A Case Report.
Transplantation proceedingsImproving quality of care in systemic sclerosis.
Best practice & research. Clinical rheumatologyComparing Novel and Legacy Health Assessment Questionnaire Scoring Methods: A Scleroderma Patient-centered Intervention Network Cohort Study.
The Journal of rheumatologyFactor Analysis to Determine Subgroups of Gastrointestinal Symptoms in Systemic Sclerosis.
The Journal of rheumatologyPrognostic and Functional Trajectories in Idiopathic Pulmonary Fibrosis and Systemic Autoimmune Rheumatic Disease-Associated Interstitial Lung Disease: Insights From an Italian Multicenter Cohort.
The Journal of rheumatologyGastrointestinal involvement in systemic sclerosis: A spotlight on small bowel involvement, complications and management.
Best practice & research. Clinical rheumatologyHigh-dose chemotherapy and transplantation of autologous CD34+ selected stem cells for progressive systemic sclerosis adjusted to cardiac and lung manifestation: An open-label, non-inferiority phase 2 trial.
Joint bone spineTh17-related genes PGAP1 and TMBIM1 serve as potential diagnostic and predictive biomarkers in systemic sclerosis: bioinformatic identification and murine model validation.
Clinical rheumatologyIL-35 enhances IL-10⁺ breg-mediated immunoregulation and attenuates inflammation and fibrosis in systemic sclerosis.
Arthritis research & therapyFrom inhibition to regulation: serpins in health and disease.
Biomedical journalRisk stratifying systemic sclerosis-related pulmonary hypertension by left atrial strain.
Rheumatology (Oxford, England)Plant-Derived Agents and Systemic Sclerosis: A Systematic Review of Therapeutic Potential and Molecular Mechanisms.
Current issues in molecular biologyAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Autologous stem cell transplant for severe, progressive juvenile systemic sclerosis.
- Automatic assessment of lung involvement in systemic sclerosis using deep learning.Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences· 2026· PMID 41858826mais citado
- When atrioventricular block paves the way to a more severe diagnosis: a case report.
- Mechanical gating of tendon fibrogenic transcription in systemic sclerosis.
- Resting Oxygen Consumption Estimates in Scleroderma Can Lead to Underestimation of Cardiac Output.
- Tocilizumab as a Potential Alternative Therapy for Acute Exacerbation of Systemic Sclerosis-Associated Interstitial Lung Disease in a Dialysis Patient Following Immunodeficiency-Associated Lymphoproliferative Disorders.
- Screening, monitoring and treatment of interstitial lung disease in systemic sclerosis.
- Physical rehabilitation interventions for hand function in people with systemic sclerosis.
- Clinical relevance of specific and non-specific autoantibodies in systemic sclerosis without overlap features.
- The Effect of Adding Paraffin Bath to the Therapeutic Hand Exercise Programme on Hand Functions in Patients with Systemic Sclerosis: A Randomized, Controlled, Single-Blind Study.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:90291(Orphanet)
- MONDO:0005100(MONDO)
- Esclerose Sistemica(PCDT · Ministério da Saúde)
- GARD:9748(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q5340515(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
