A esclerodermia é uma doença autoimune rara do tecido conjuntivo caracterizada pelo endurecimento anormal da pele e, às vezes, de outros órgãos. É classificada em duas formas principais: esclerodermia localizada e esclerose sistêmica (ES), esta última compreendendo três subgrupos; ES cutânea difusa (SSc), ES cutânea limitada (SSc) e ES limitada (ESl).
Introdução
O que você precisa saber de cara
A esclerodermia é uma doença autoimune rara do tecido conjuntivo caracterizada pelo endurecimento anormal da pele e, às vezes, de outros órgãos. É classificada em duas formas principais: esclerodermia localizada e esclerose sistêmica (ES), esta última compreendendo três subgrupos; ES cutânea difusa (SSc), ES cutânea limitada (SSc) e ES limitada (ESl).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 63 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 157 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: 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
Variantes genéticas (ClinVar)
57 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 1 variantes classificadas pelo 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)
🇧🇷 Atendimento SUS — Esclerodermia
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
🟢 Recrutando agora
4 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
779 ensaios clínicos encontrados, 9 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 5.825
Autologous stem cell transplant for severe, progressive juvenile systemic sclerosis.
A esclerose sistêmica juvenil (jSSc) é uma doença autoimune rara e grave em crianças/adolescentes, caracterizada por endurecimento da pele, danos a órgãos e opções terapêuticas subótimas. Diante da limitação dos tratamentos convencionais, este estudo de caso relata uma melhora clínica promissora em um paciente de 17 anos com jSSc refratária após um transplante autólogo de células-tronco. Isso sugere uma potencial nova via de tratamento para casos graves e resistentes.
🇧🇷 traduzidoAutomatic assessment of lung involvement in systemic sclerosis using deep learning.
Este estudo utiliza inteligência artificial (deep learning) para avaliar automaticamente o envolvimento pulmonar em pacientes com esclerodermia sistêmica, aprimorando a precisão diagnóstica em tomografias e minimizando erros humanos. A tecnologia identifica e quantifica as áreas de dano pulmonar, como reticulação e opacidades em vidro fosco, oferecendo aos médicos uma medida objetiva e detalhada da extensão da doença para um acompanhamento e tratamento mais eficazes dos pacientes.
🇧🇷 traduzidoResting Oxygen Consumption Estimates in Scleroderma Can Lead to Underestimation of Cardiac Output.
Este estudo alerta que as estimativas padrão do consumo de oxigênio em repouso (rVO2), usadas para calcular a função cardíaca (débito cardíaco) em pacientes com esclerodermia, podem ser imprecisas. Para médicos e pacientes, isso significa que essas estimativas podem levar a uma subestimação do quão bem o coração está bombeando, resultando em diagnósticos errados e impactando o tratamento de problemas cardíacos. A medição direta do rVO2 é, portanto, recomendada para maior precisão diagnóstica.
🇧🇷 traduzidoThe Th17/Treg axis: a key to understanding and treating autoimmune disorders.
Em doenças autoimunes como a esclerodermia, o sistema imunológico ataca o próprio corpo devido a um desequilíbrio entre as células T Th17 (promotoras de inflamação) e as Treg (reguladoras), resultando em inflamação crônica e danos aos tecidos. Compreender e restaurar este balanço é crucial, pois os níveis dessas células podem indicar a gravidade da doença e a eficácia do tratamento, servindo como um alvo terapêutico fundamental para otimizar abordagens clínicas atuais e futuras.
🇧🇷 traduzidoIdentification of key macrophage-related genes in systemic sclerosis-associated interstitial lung disease based on single-cell and bulk transcriptomic data.
Focado na grave doença pulmonar intersticial associada à esclerose sistêmica (SSc-ILD), a principal causa de morte em pacientes com esclerose sistêmica e sem tratamentos eficazes, este estudo identificou os genes **ARG2, ELF3 e NKX2-1** como fatores-chave nos mecanismos relacionados aos macrófagos da doença. Para pacientes e médicos, esses achados são cruciais, pois destacam esses genes como potenciais alvos terapêuticos, com resultados de predição de drogas já sugerindo possíveis interações com medicamentos existentes, o que pode abrir caminho para o desenvolvimento de novas e mais eficazes opções de tratamento.
🇧🇷 traduzidoPublicações 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.
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.
Systemic sclerosis-related digital ulcers: Current understanding and updated management approaches- a primer for clinicians.
📚 EuropePMC9.750 artigos no totalmostrando 198
Autologous stem cell transplant for severe, progressive juvenile systemic sclerosis.
Stem cells translational medicineDefinition, diagnosis, treatment for scleroderma primary heart involvement.
Best practice & research. Clinical rheumatologyA practical clinical approach to the diagnosis and management of morphea (localized scleroderma).
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 SciencesPrevalence of comorbidities and medication use in progressive systemic sclerosis in northeast Mexico.
Reumatologia clinicaImplications of Dermatologic Disorders in Facial Cosmetic Surgery: A Systematic Review.
Annals of plastic surgeryCircumscribed Morphea Successfully Treated With Excimer Laser: Analysis of Histopathological Changes.
The Journal of dermatologyStrabismus as the Initial Manifestation of En Coup De Sabre in Adult Patients.
Neuro-ophthalmology (Aeolus Press)The 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 diseasesPerformance 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 rheumatologyNailfold capillaroscopy improves cardiovascular risk stratification in SSc: an adjustment of the SCORE2 algorithm.
Rheumatology (Oxford, England)Fibrosis-memory is mediated by IL-3-producing T cells and drives progression of fibrosis.
The Journal of clinical investigationChronic graft versus host disease in upper limb: Calling upper limb rehabilitation specialists for improving outcomes in patients.
Clinical hematology internationalOptical 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 rheumatismTrends and outcomes of lung transplant listings for connective tissue disease-associated interstitial lung disease (CTD-ILD): A 20-Year analysis from the organ procurement and transplantation network database.
Seminars in arthritis and rheumatismExploring Disease-Specific Risk Factors for Vertebral Fractures in Systemic Sclerosis: Insights from the ScleroRER Study Group.
Journal of clinical medicineNailfold capillaroscopy and organ involvement in systemic sclerosis: a systematic review.
Best practice & research. Clinical rheumatologyResting Oxygen Consumption Estimates in Scleroderma Can Lead to Underestimation of Cardiac Output.
Pulmonary circulationAnalysis of fungal community structure and co-occurrence networks across vegetation types in volcanic lava habitats.
Frontiers in fungal biologyHuman 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 rheumatologyIntravenous immunoglobulin for the treatment of morphea in pediatric patients: A case series.
JAAD case reportsCommunity-based management of gastrointestinal symptoms at end-of-life in scleroderma using subcutaneous medication: a case report.
Annals of palliative medicineThe Th17/Treg axis: a key to understanding and treating autoimmune disorders.
Open life sciencesA novel microneedle-based delivery of NRF2-overexpressing exosomes for scleroderma therapy.
International journal of biological macromoleculesNintedanib Attenuates Profibrotic Gene Expression in a 3D Organotypic Culture Model of Systemic Sclerosis Skin Fibrosis.
The Journal of investigative dermatologyLongitudinal Assessment of the Juvenile Systemic Sclerosis Severity Score: Application in the National Registry for Childhood Onset Scleroderma.
ACR open rheumatologyA retrospective analysis of clinical characteristics of systemic sclerosis-associated interstitial lung disease.
MedicineCase 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 pharmacologyIdentification of key macrophage-related genes in systemic sclerosis-associated interstitial lung disease based on single-cell and bulk transcriptomic data.
PloS oneThe 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 diseaseNot all small bowel pneumatosis is ischaemia: a case-based systematic review of non-ischaemic causes.
Annals of the Royal College of Surgeons of EnglandRecent advances in the heart and soul of primary cardiac involvement and cardiovascular disease in systemic sclerosis.
Best practice & research. Clinical rheumatologyIll by mouth? Patients' experiences of the oral and dental manifestations of scleroderma.
Health (London, England : 1997)[Shulman syndrome: An atypical presentation of a rare disease].
Revista medica del Instituto Mexicano del Seguro SocialInjectable platelet-rich fibrin as a valid therapeutic option for inactive linear morphea (en coup de saber): report of cases.
The Journal of dermatological treatmentGeneralized Morphea Successfully Treated With Tripterygium Glycosides and Hydroxychloroquine: A Case Report and a Systematic Review.
Cureus2025 expert consensus on the use of parenteral prostanoids in incident and prevalent PAH patients: The Italian perspective.
Vascular pharmacologyEvaluation of non-invasive techniques for the diagnosis of pulmonary complications in systemic sclerosis and scleroderma-like overlap syndromes: role of lung ultrasound.
Journal of autoimmunityThe Incidence of Painful Neuroma After Digital Amputation Due to Trauma Versus Chronic Ischemia.
Hand (New York, N.Y.)Outcomes 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 immunologyScleroderma clinical trials consortium classification criteria for systemic sclerosis heart involvement.
Rheumatology (Oxford, England)Successful tocilizumab-based combination therapy for a case of rapidly progressive adult deep morphea with multiple antiphospholipid antibodies: a case report and literature review.
Frontiers in immunologyA 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.
ReumatismoTemporomandibular joint disorders in patients with systemic sclerosis: a cross-sectional study.
BMC 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 reviewsReplay: Comparative Study of Adipose-Derived Stem Cells from Localized Scleroderma Patients and Healthy Donors in Treating Skin Fibrosis.
Plastic and reconstructive surgeryExtended temporomandibular joint prosthetic reconstruction of total condylysis in the setting of severe scleroderma: a case report.
Oral and maxillofacial surgeryShared governance of international myositis networks.
Clinical and experimental rheumatologyLong-term follow-up of linear scleroderma en coup de sabre in children with central nervous system involvement.
Frontiers in immunologyA 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 reportsIntegrating 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 reviewsEvaluating 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 sciencesReduced 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 immunologyCarbon dioxide vs. warm-water hand baths in systemic sclerosis with secondary Raynaud's syndrome - A capillaroscopy centered randomized controlled trial.
Microvascular researchDrug development in systemic sclerosis: novel therapeutics to watch.
Expert review of clinical immunologyPrevalence and clinical relevance of systematically tested antimitochondrial antibodies in systemic sclerosis.
Arthritis research & therapyCytometry-based blood immune cell markers associated with clinical outcomes in systemic sclerosis: protocol for a systematic review.
BMJ openMechanistic basis for the antifibrotic actions of cAMP-based therapies.
European respiratory review : an official journal of the European Respiratory SocietyManagement of pulmonary arterial hypertension in systemic sclerosis: from classical treatments to new horizons.
European respiratory review : an official journal of the European Respiratory SocietyAssessing the structural validity of the SCL-Dep6 depression subscale using rasch analysis.
Journal of affective disordersSystemic 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.
CureusPrognostic biomarkers for idiopathic pulmonary fibrosis: findings from ISABELA clinical trials.
ERJ open researchA potent inhibitor of PAI-1, MDI-2517, mitigates disease severity in a preclinical systemic sclerosis model.
JCI insightUstekinumab-associated morphoea: systematic review of the literature and a real-world case.
Drugs in contextFacial Swelling in a Young Adult With Type 1 Diabetes: Morbihan Disease as a Scleroderma Mimic.
CureusAn 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 rheumatologyAdministration of Hormone Replacement Therapy Containing Estrogen and Development of Autoimmune Associated Vocal Fold Lesions in Women.
Journal of voice : official journal of the Voice FoundationSecond 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 rheumatismQuantifying the Prevalence of Disorders of Gut Brain Interaction in Systemic Sclerosis.
Neurogastroenterology and motilityEvaluation of serial cardiovascular magnetic resonance monitoring and immunosuppressive therapy in predicting outcomes in systemic sclerosis.
RMD openDevelopment of enzyme-linked immunosorbent assays for the detection of myositis-specific autoantibodies against signal recognition particle, nuclear matrix protein 2, and small ubiquitin-like modifier activating enzyme.
Modern rheumatologyAnalysis of the Dynamics of Clinical Parameters and Serum Angiogenic Factors in Systemic Sclerosis Patients Undergoing Tocilizumab Treatment.
Experimental dermatologyAge 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 diseaseReactive 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)Venous Abnormalities in Biopsy-confirmed Lipodermatosclerosis.
Advances in skin & wound careMulti-omics profiling of chronic immune-mediated skin diseases: SKINERGY protocol and strategic evaluation.
Journal of the European Academy of Dermatology and Venereology : JEADVGain 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 ethnopharmacologyThe 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 diseasesAtypical Presentation of Morphoea in an Elderly Male: Diagnostic Challenges in the Absence of Autoantibodies and Malignancy.
CureusJuvenile systemic sclerosis.
Best practice & research. Clinical rheumatologyTPI1 and TPM4 are strong candidate RNA biomarkers for systemic sclerosis.
Arthritis research & therapySpectroscopic and machine learning approaches for clinical subtyping in systemic sclerosis.
Scientific reportsComparing 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 rheumatologyGastrointestinal involvement in systemic sclerosis: A spotlight on small bowel involvement, complications and management.
Best practice & research. Clinical rheumatologyTreatment of juvenile localized scleroderma with upadacitinib: A case series.
Journal of the European Academy of Dermatology and Venereology : JEADVTh17-related genes PGAP1 and TMBIM1 serve as potential diagnostic and predictive biomarkers in systemic sclerosis: bioinformatic identification and murine model validation.
Clinical rheumatologyRisk stratifying systemic sclerosis-related pulmonary hypertension by left atrial strain.
Rheumatology (Oxford, England)Fc-enhanced anti-CCR6 antibody elicits robust therapeutic effects across multiple autoimmune diseases.
Frontiers in immunologyRenal Vascular Affection in Systemic Sclerosis Patients and Possible Correlation with Peripheral Vascular Involvement Assessed by Nailfold Capillaroscopy.
Mediterranean journal of rheumatologyEvaluation of a screening algorithm to detect systemic sclerosis-related myopathy.
Rheumatology (Oxford, England)Methotrexate and Mycophenolate Mofetil and Clinical Response in Juvenile Localized Scleroderma.
JAMA dermatologyAssociation of MMP9 and NOS3 Polymorphisms with Distinct Clinical Forms of Juvenile Scleroderma and Characteristics of Humoral Immunity.
International journal of molecular sciencesConnective Tissue Disease-Associated Pulmonary Arterial Hypertension: Current Therapeutic Strategies and Future Prospects.
BiomoleculesSerum CCL18 May Reflect Multiorgan Involvement with Poor Outcome in Systemic Sclerosis.
BiomoleculesJAK inhibitors for the treatment of juvenile localized scleroderma: a case report and literature review.
Pediatric rheumatology online journalClose association of kinesiophobia with physical performance in patients with systemic sclerosis.
Rheumatology internationalPrevalence and severity of complicated Raynaud's phenomenon in limited and diffuse systemic sclerosis: a multicenter study in Iraq.
ReumatologiaBotulinum neurotoxin type A1 alleviates bleomycin-induced skin fibrosis by inhibiting phosphatidylinositol 3-kinase-protein kinase B (PI3K-Akt) pathway and associated cell cycle and proliferation.
The Journal of investigative dermatologyGastrointestinal histology of systemic sclerosis: A systematic review.
Autoimmunity reviewsPatient partnership model in rare and complex rheumatological conditions: research and beyond in European Reference Network ReCONNET.
The Lancet. RheumatologyStrengthening rheumatology research through meaningful engagement of people with lived experience.
The Lancet. RheumatologyDevelopment of a tool to map SSc pain sources, characteristics and management experiences: the SPIN Pain Assessment Tool.
Rheumatology (Oxford, England)Linear scleroderma 'en coup de sabre'.
Practical neurologyLike an Osteophyte. . . or Worse? A Curious Case of Dysphagia in a Patient With Scleroderma.
Ear, nose, & throat journalMacrophages Are Critical Inducers of Bleomycin-Induced Fibrosis in a Systemic Scleroderma Model.
The American journal of pathologySurgical Management of Systemic Sclerosis: Review of Literature.
Current rheumatology reviewsForced vital capacity as a survival predictor in systemic sclerosis-associated pulmonary hypertension.
Therapeutic advances in musculoskeletal diseaseEsomeprazole inhibits proliferation of scleroderma fibroblasts via cell cycle regulation.
Frontiers in pharmacologyBeyond the breast: Mammographic manifestations of systemic disease.
Current problems in diagnostic radiologyAutoantibodies as Precision Tools in Connective Tissue Diseases: From Epiphenomenon to Endophenotype.
Antibodies (Basel, Switzerland)Differential Item Functioning on the Patient Health Questionnaire 8 by Disease Subtype, Language, Sex, and Age Among People With Systemic Sclerosis: A Scleroderma Patient-Centered Intervention Network Cohort Study.
Arthritis care & researchSystemic Sclerosis-Related Intestinal Pseudo-Obstruction Mimicking Mechanical Small Bowel Obstruction.
CureusContemporary 0.55 T MRI to visualize interstitial lung disease - An exploratory study.
European journal of radiology openThe added value of cardiac MRI in paediatric rheumatologic disorders: a preliminary single-centre experience.
Cardiology in the youngThe incidence of interstitial lung disease in patients with systemic sclerosis: rate, risk factors and prognostic implications in a EUSTAR cohort analysis (CP 133).
Annals of the rheumatic diseasesDeficiency of inducible nitric oxide synthase alleviates dermal fibrosis and inflammation in scleroderma.
International immunopharmacologyAssociations with, and predictors of, progression in systemic sclerosis-related interstitial lung disease: a scoping literature review.
European respiratory review : an official journal of the European Respiratory SocietyMASLD-Induced Hyperammonemia With Atypical, Restricted Diffusion Pattern Suggesting Mixed Metabolic and Hypoxic-Anoxic Injury.
HCA healthcare journal of medicineTofacitinib treatment in a girl with localized scleroderma of the head and face: a case-based review.
Rheumatology internationalSkin Cancer Arising in Morphea: A Systematic Review.
Journal of cutaneous medicine and surgeryA Case of Mandibular Osteomyelitis Occurring in a Patient With Parry-Romberg Syndrome.
CureusExploring the Association Between Autoimmune and Inflammatory Diseases and Uveitis.
American journal of ophthalmologySoluble CD13 in systemic sclerosis: clinical observations and transcriptomic insights from peripheral blood.
Arthritis research & therapyTemporal Dynamics of Pulmonary Fibrosis and Immune Dysregulation in a Collagen V-Driven Systemic Sclerosis Model.
International journal of molecular sciencesSystemic Sclerosis-Associated ILD: Insights and Limitations of ScleroID.
Diagnostics (Basel, Switzerland)A Case Report on a Challenge From Diagnosis to Treatment: Interstitial Lung Disease As the First Manifestation of Systemic Sclerosis Sine Scleroderma.
CureusHeart Transplantation in Patients With Systemic Sclerosis.
Clinical transplantationClinical Applications of Autologous Fat Grafting in Pathological Hand Conditions.
The journal of hand surgery Asian-Pacific volumeDisease Course and Risk of Relapse in Juvenile Localized Scleroderma: A Single-Center Retrospective Study.
International journal of rheumatic diseasesClinical predictors of mortality in critically ill systemic sclerosis patients: a 6-year retrospective cohort study.
Rheumatology (Oxford, England)CD19 CAR-T cells for treatment-refractory autoimmune diseases: the phase 1/2 CASTLE basket trial.
Nature medicineTranscriptomic analysis under the stress of insecticides and the functional role of three genes related to host finding behavior of Scleroderma sichuanensis.
Comparative biochemistry and physiology. Part D, Genomics & proteomicsInterstitial lung disease in systemic sclerosis-a retrospective cross-sectional study in Taiwan.
Clinical rheumatologyMesenchymal stromal cells ameliorate systemic sclerosis-interstitial lung disease via PD-1/PD-L1 signalling axis.
RMD openCombination of Oral Hydroxychloroquine and Topical Pimecrolimus 1% for Linear Morphea in Breastfeeding: A Rare Case with Favorable Outcome.
International journal of women's healthNailfold videocapillaroscopy in the assessment of juvenile connective tissue diseases.
Clinical and experimental rheumatologyDeficiency of NRF2 aggravates BLM-induced systemic sclerosis-associated fibrosis and inflammation in mice.
International immunopharmacologyExpression and clinical significance of fatty acid metabolism-related genes in PBMCs of SSc patients.
Clinical rheumatologySkin-derived myeloid precursors and joint-resident fibroblasts spread psoriatic disease from skin to joints.
Nature immunologySubclinical right ventricular dysfunction in juvenile systemic sclerosis: a cross-sectional echocardiographic study.
ARP rheumatologyGangrenous Digital Ischemia Unmasking HIV Infection in Untreated Systemic Sclerosis.
CureusThe Evolution in the Cutaneous and Gastrointestinal Pathology of Pediatric Kohlmeier-Degos Disease Under Combined Modality Therapy.
The American Journal of dermatopathologyParry-Romberg Syndrome Associated With En Coup De Sabre: A Clinical Case Report.
CureusMYCOPHENOLATE MOFETIL TREATMENT REDUCES THE RISK OF TREATMENT ESCALATION DUE TO VASCULAR COMPLICATIONS IN LIMITED CUTANEOUS SYSTEMIC SCLEROSIS: EMULATION OF A TARGET TRIAL FROM ITALIAN RHEUMATOLOGY SOCIETY SPRING REGISTRY.
Arthritis care & researchChromosomal Instability and Telomere Attrition in Systemic Sclerosis: A Historical Perspective.
GenesAutoimmune disorders as predictive factors for oral lichen planus and malignancy risk: real-world data analysis.
Oral surgery, oral medicine, oral pathology and oral radiologyHand function impairment in Systemic Sclerosis: determinants through investigations of clinical, radiographic and ultrasonographic features.
Seminars in arthritis and rheumatismPitfalls in the treatment of keloidal scleroderma.
Internal medicine journalAntineutrophil Cytoplasmic Antibody-Associated Vasculitides in Systemic Sclerosis: A Unique Clinical Overlap With Significant Implications for Treatment and Outcomes.
Arthritis & rheumatology (Hoboken, N.J.)Single-Cell Profiling Reveals Divergent Mechanisms of Fibrosis in Localized Scleroderma and Systemic Sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)Effects of multimodal nutritional intervention on nutritional status and clinical outcomes in patients with systemic sclerosis: study protocol for a randomised controlled trial.
BMJ openFrom the grey area of pre-systemic sclerosis to very early disease and irreversible tissue damage: the challenge of defining at-risk patients for future preventive trials in systemic sclerosis.
Annals of the rheumatic diseasesAtorvastatin alleviates the progression of fibrosis in experimental systemic sclerosis: Binding to macrophage CD14 to regulate TLR4/p65 or directly modulating fibroblast Smad3 signaling.
Free radical biology & medicineSystemic Sclerosis-Recent Advances in Diagnosis and Management.
British journal of hospital medicine (London, England : 2005)Systemic Sclerosis-Associated Interstitial Lung Disease: Improved Understanding and Advances in Management.
British journal of hospital medicine (London, England : 2005)Work participation and work disability in Belgian patients with systemic sclerosis.
Rheumatology (Oxford, England)Integration of natural language models in the diagnosis of systemic autoimmune diseases: validation of GPT-4 in a tertiary care center.
Revista clinica espanolaThe altered T cell landscape in Systemic Sclerosis patients is characterized by dysfunctional type 1 immunity.
bioRxiv : the preprint server for biologyUnderrepresentation of Black participants in systemic sclerosis clinical trials: the U.S.A. and southeast region.
Clinical rheumatologyThe efficacy and safety of tocilizumab in juvenile localised scleroderma patients: a retrospective cohort study in Eastern China.
Advances in rheumatology (London, England)Radiographic Fibrosis Extent Identifies Immunomodulatory Treatment Response in Hypersensitivity Pneumonitis.
Chest[Eosinophilic fascitis: A case report and literature review].
Revista medica del Instituto Mexicano del Seguro SocialPhosphodiesterase-4 inhibition in localized scleroderma: A case series of topical roflumilast.
SAGE open medical case reportsSgA1d, an acid lipase from venom of the ectoparasitoid Scleroderma guani involved in the regulation of host lipid metabolism.
Pest management scienceMultimodal analyses of early, untreated systemic sclerosis skin identify a proinflammatory vascular niche of macrophage-fibroblast signaling.
JCI insightTrigeminal neuropathy as the first symptom of mixed connective tissue disease: case report and literature review.
Quintessence international (Berlin, Germany : 1985)Serum profiling of uPA, PAI-1, and suPAR in systemic sclerosis: a preliminary study on analytical aspects and associations with microvascular and fibrotic manifestations.
Frontiers in immunologyGut microbiome-metabolome dysregulation in systemic sclerosis: a multi-omics study.
Rheumatology (Oxford, England)Successful Treatment of Refractory Severe Localized Scleroderma With Anifrolumab.
JAMA dermatologyHidradenitis Suppurativa and Smoking, Obesity, Psoriasis, Inflammatory Bowel Disease, and Systemic Sclerosis: Results From A 2-Sample Mendelian Randomization Study.
JAMA dermatologyAutomated lung texture analysis for assessing interstitial lung disease in systemic sclerosis: Diagnostic accuracy in photon-counting-detector and conventional energy-integrating-detector CT.
European journal of radiology[POEMS syndrome misdiagnosed as systemic sclerosis: A case report].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences[Rheumatic disease spectrum and immunological profile of anti-PM/Scl antibodies in idiopathic inflammatory myopathies].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesAssociações
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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
- Resting Oxygen Consumption Estimates in Scleroderma Can Lead to Underestimation of Cardiac Output.
- The Th17/Treg axis: a key to understanding and treating autoimmune disorders.
- Identification of key macrophage-related genes in systemic sclerosis-associated interstitial lung disease based on single-cell and bulk transcriptomic data.
- 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.
- 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.
- Systemic sclerosis-related digital ulcers: Current understanding and updated management approaches- a primer for clinicians.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:801(Orphanet)
- MONDO:0019340(MONDO)
- Esclerose Sistemica(PCDT · Ministério da Saúde)
- GARD:18705(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q958797(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
