A Esclerose Sistêmica Cutânea Difusa (ESCD) é um subtipo de Esclerose Sistêmica (ES) caracterizado pelo endurecimento da pele no tronco e nas extremidades. Essa forma da doença tem uma alta e precoce incidência de acometimento espalhado (atingindo vários órgãos), incluindo: doença pulmonar intersticial (um tipo de problema nos pulmões), insuficiência renal (problemas graves nos rins, que produzem pouca urina), doença gastrointestinal difusa (problemas generalizados no sistema digestivo) e acometimento do músculo do coração.
Introdução
O que você precisa saber de cara
A Esclerose Sistêmica Cutânea Difusa (ESCD) é um subtipo de Esclerose Sistêmica (ES) caracterizado pelo endurecimento da pele no tronco e nas extremidades. Essa forma da doença tem uma alta e precoce incidência de acometimento espalhado (atingindo vários órgãos), incluindo: doença pulmonar intersticial (um tipo de problema nos pulmões), insuficiência renal (problemas graves nos rins, que produzem pouca urina), doença gastrointestinal difusa (problemas generalizados no sistema digestivo) e acometimento do músculo do coração.
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
+ 8 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 26 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
5 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial, Not applicable.
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.
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
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.
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
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.
Variantes genéticas (ClinVar)
76 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)
🇧🇷 Atendimento SUS — Esclerose sistêmica cutânea difusa
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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Ensaios em destaque
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13 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
83 ensaios clínicos encontrados, 19 ativos.
Publicações mais relevantes
Cytometry-based blood immune cell markers associated with clinical outcomes in systemic sclerosis: protocol for a systematic review.
Systemic sclerosis (SSc) is a rare autoimmune disease characterised by skin and organ fibrosis, vasculopathy and immune dysregulation. Given the disease heterogeneity and severity, accurate prognostic and predictive markers are needed. Blood immunophenotyping by flow or mass cytometry offers a promising non-invasive approach to identify immune signatures associated with disease subtypes, complications (eg, interstitial lung disease, scleroderma renal crisis, digital ulcers) and treatment responses. However, findings remain inconsistent and lack clinical standardisation. This systematic review aims to identify cytometry-based blood immune markers associated with clinical outcomes in SSc. A comprehensive search will be conducted on three databases: PubMed, Web of Science and Cochrane Library, from their inception to the date of the final search (21 December 2025). Data will be extracted and analysed using a predefined charting form. Studies published in English or French reporting the use of flow or mass cytometry for peripheral blood cells phenotyping in adults with SSc will be included. Comparators will include healthy controls, other autoimmune diseases, disease severity groups and treatment response subgroups. The primary outcome will be the association with the diffuse cutaneous subtype, while secondary outcomes will include overall survival and disease-specific mortality, organ involvement, progression of the disease and treatment response. Ethical approval is not required as this review is an analysis of published scientific literature and does not involve patients. The results of this systematic literature review will be submitted for publication in a peer-reviewed journal and presented at relevant conferences. CRD420250644594.
Fetal and maternal outcome in the pregnancies of patients with systemic sclerosis and very early diagnosis of systemic sclerosis in France: a prospective study.
Prospective data on pregnancies in systemic sclerosis are scarce. We aimed to examine the frequency of adverse pregnancy outcomes and maternal disease progression in systemic sclerosis, as well as the factors that predict these events. In this analysis, we studied pregnant women with systemic sclerosis (American College of Rheumatology-European League Against Rheumatism 2013 classification) or with Very Early Diagnosis of Systemic Sclerosis (VEDOSS criteria) included in the GR2 French prospective study. Frequency of composite adverse pregnancy outcomes (preterm birth at 34 weeks or less, placental insufficiency complications, small for gestational age, or fetal or neonatal death) and maternal disease course were the primary objectives. The secondary objectives were to assess other complications related to pregnancy (including delivery outcomes and postpartum complications) and compare these results with outcomes for age-matched controls from the French perinatal survey (ENP) 2016 (ie, general population), and to identify predictive factors associated with composite adverse pregnancy outcomes and maternal disease course using univariate analysis. Between May 1, 2014, and Dec 27, 2020, we included 58 pregnancies (in 52 women), with 53 (91·4%) resulting in livebirths. Of the 53 ongoing pregnancies beyond 22 weeks of gestation, 14 (26·4%) had a composite adverse pregnancy outcome, including two (3·8%) preterm deliveries at 34 weeks of gestation or less, 12 (22·6%) placental insufficiency complications (pre-eclampsia or fetal growth restriction), and six (11·3%) small for gestational age. Among the 53 pregnancies, six (11·3%) severe postpartum haemorrhage events occurred. When compared with the 2016 ENP survey results, pre-eclampsia (seven [13·2%] of 53 vs 16 [3·0%] of 530, p=0·0010, preterm birth before 37 weeks of gestation (seven [13·2%] of 53 vs 31 [5·8%] of 530, p=0·047), birthweight of less than 2500 g (11 [21·1%] of 52 vs 23 [4·3%] of 530, p<0·0001), and severe postpartum haemorrhage (six [11·3%] of 53 vs seven [1·4%] of 516, p=0·0001) were more frequent than in the general population. No factors were significantly associated with the composite adverse pregnancy outcome in univariate analysis. Systemic sclerosis or VEDOSS worsened in 23 (39·7%) of 58 pregnancies, mainly during the postpartum period. In the univariate analysis, diffuse cutaneous systemic sclerosis (odds ratio 3·7 [95% CI 1·1-12·4]) and previous cutaneous vascular involvement (3·7 [1·2-11·5]) were associated with maternal disease progression, whereas the presence of anticentromere antibodies was inversely associated with stable disease (0·2 [0·1-0·8]). Despite 53 (91·4%) of 58 livebirths, systemic sclerosis pregnancies were associated with higher rates of adverse pregnancy outcomes and severe postpartum haemorrhage. Disease worsened in 23 (39·7%) of 58 pregnancies, particularly during the postpartum period, especially in women with diffuse cutaneous systemic sclerosis, previous cutaneous vascular involvement, and antibodies other than anticentromere. Lupus France, Association des Sclérodermiques de France, Association Gougerot Sjögren, Association Francophone Contre la Polychondrite Chronique Atrophiante, AFM-Telethon, Société Nationale Française de Médecine Interne, Société Française de Rhumatologie, Cochin Hospital, French Health Ministry, Fondation for Research in Rheumatology, Association Prix Véronique Roualet, Union Chimique Belge.
Efficacy and Safety of Tofacitinib Compared to Cyclophosphamide in Early Diffuse Cutaneous Systemic Sclerosis: A Randomized Controlled Trial.
This study aims to determine the efficacy of tofacitinib in the treatment of early diffuse cutaneous systemic sclerosis. This open-label randomized controlled clinical trial was conducted at the Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University, Dhaka. The trial was registered at ClinicalTrials.gov (identifier: NCT06044844). Consecutive sampling was used in this study. Forty-six patients were randomized to groups A and B using block randomization, with 23 patients in each group. In group A, tofacitinib (5 mg) was administered twice daily. Group B received cyclophosphamide (500 mg/m² monthly). Primary efficacy was assessed by the change in the Modified Rodnan Skin Score (mRSS) from baseline after 24 weeks. Secondary efficacy was assessed by the change in the Disease Activity Score for 28 joints (DAS28) in response to changes in C-reactive protein (CRP) level and erythrocyte sedimentation rate (ESR). The Bangla version of the Health Assessment Questionnaire-Disability Index (B-HAQ) response from baseline to 24 weeks was analyzed. Oral prednisolone (≤10 mg/day), calcium channel blockers, and phosphodiesterase 5 inhibitors (sildenafil and tadalafil) were administered. Follow-up was performed at four, 12, and 24 weeks. History, physical examination, and relevant investigations were used to assess adverse effects. Changes in acute-phase reactants and composite measures within the groups from baseline to 24 weeks were also analyzed. Adverse effects were assessed through patient history, physical examination, and relevant investigations. Per-protocol analysis showed a significantly greater reduction in mRSS in group A compared with group B at 12 weeks (7.0 ± 2.89 vs. 5.26 ± 2.42; p = 0.03) and 24 weeks (10.17 ± 2.92 vs. 8.0 ± 4.08; p = 0.04). DAS28-ESR and DAS28-CRP reductions were significant between the groups from baseline to weeks 12 and 24 (p < 0.05). The reduction in functional status (measured by B-HAQ) was 2.11 ± 4.91 and 0.96 ± 0.53 in groups A and B, respectively (p = 0.43). FVC change was 9.17 ± 8.33 in group A and 3.43 ± 8.1 in group B. Within the other core set of outcomes, composite measures were significantly improved in group A. Two patients (8.7%) in group A and six patients (17.4%) in group B developed nausea. Two patients (8.7%) in both groups developed respiratory tract infections and urinary tract infections. Taeniasis developed in three patients (13%) in group B. Two cyclophosphamide-treated patients (8.7%) developed hemorrhagic cystitis. Tofacitinib demonstrated significant efficacy in reducing skin thickness (mRSS) in early dcSSc, showing both numerical and statistical advantages over cyclophosphamide at 24 weeks, along with a more favorable safety profile. These findings support further evaluation of tofacitinib as a potential therapeutic option in this patient population.
A retrospective analysis of clinical characteristics of systemic sclerosis-associated interstitial lung disease.
Systemic sclerosis (SSc) is a rare, complex, chronic, progressive, severe, often life-threatening, fibrosing, heterogeneous autoimmune connective tissue disease with immune dysfunction and limited treatment options. Interstitial lung disease (ILD) is common in SSc. To study the clinical characteristics of systemic sclerosis-associated interstitial lung disease (SSc-ILD) will provide evidence for the early diagnosis, treatment decision-making, and prognosis of SSc-ILD patients. One hundred and seventy-five patients with SSc were retrospectively enrolled and divided into SSc-ILD group and SSc-non-ILD group. The general clinical characteristics, autoantibodies, laboratory test, thorax high-resolution computed tomography (HRCT), pulmonary function testing, stratification, and treatment options were compared between the 2 groups. The risk factors of SSc-ILD were also analyzed. One hundred and twenty-four SSc-ILD patients (70.9%) and 51 SSc-non-ILD patients (29.1%) were in 175 SSc patients. Raynaud phenomenon, skin swelling, and joint involvement were the top 3 initial symptoms. Sixty-seven SSc-ILD patients (54.1%) showed new onset or progression of ILD on HRCT, which were heterogeneous. The significantly increased indicators in SSc-ILD group were gastrointestinal involvement (P = .003), antinuclear antibody (ANA) (P = .006), anti-Scl-70 (P <.001), anti-SSA (P = .008), and rheumatoid factor (P = .04), erythrocyte sedimentation rate (ESR) (P = .005), CRP (P = .000), IL-6 (P = .001), globulin (P = .006), IgG (P = .002), IgA (P = .001), while the decreased indicators were mean corpusular hemoglobin concerntration (P = .036), albumin (P = .046), liver dysfunction (P = .018), and FVC% (P = .006). Positive anti-Scl-70 antibody and decreased FVC% were independent risk factors for SSc-ILD. The incidence of ILD occurrence and progression was significantly higher in patients with anti-Scl-70 antibody, diffuse cutaneous systemic sclerosis (dcSSc) plus anti-Scl-70 antibody, ESR ≥50 mm/h, and CRP ≥5.00 mg/L (P <.05). Based on the predictive factors (gastrointestinal involvement, joint involvement, positive anti-Scl-70 antibody, liver dysfunction, duration of disease, FVC%, mean corpusular hemoglobin concerntration, ESR, CRP, IgG, IgA, globulin, and albumin), independent risk factors (anti-Scl-70 antibody and decreased FVC%), clinical phenotypic and autoantibody stratification, HRCT heterogeneity, and atypical initial symptoms, it may be seized the "window of opportunity" with early diagnosis may exist for preventing the irreversible progression of pulmonary fibrosis of SSc-ILD patients.
Crowned Dens Syndrome Mimicking Atlantoaxial Infection in a Patient With Systemic Sclerosis: A Case Report.
Crowned dens syndrome (CDS), caused by calcium pyrophosphate or hydroxyapatite deposition around the odontoid process, is an under-recognized cause of acute severe neck pain and headache. It can closely mimic septic arthritis, osteomyelitis, giant cell arteritis (GCA), polymyalgia rheumatica (PMR), or cervical spondylitis. We report a case of CDS in a patient with diffuse systemic sclerosis initially managed for presumed infection. A 55-year-old man with diffuse cutaneous systemic sclerosis on mycophenolate mofetil and methotrexate presented with severe throbbing headache, facial tenderness, and diffuse pain. CT/CTA of the head and neck were negative for vascular events; MRI revealed inflammatory changes at the atlantoaxial and atlanto-occipital joints concerning for septic arthritis/osteomyelitis with abscess formation. Broad-spectrum antibiotics were initiated. The patient's symptoms persisted despite antibiotics, prompting repeat imaging. CT demonstrated calcifications surrounding the odontoid process, raising suspicion for CDS. Colchicine and low-dose prednisone were introduced while antibiotics were continued, given ongoing concern for occult infection in an immunosuppressed host. Within 1 week, the patient experienced near-complete resolution of pain. At follow-up, he remained symptom-free, allowing reintroduction of methotrexate while mycophenolate was held. CDS should be considered in the differential diagnosis of severe headache and neck pain in rheumatology patients, particularly when imaging shows craniocervical inflammation and calcifications. CT of the odontoid is diagnostic, but MRI findings may mimic infection. Awareness of CDS is critical to avoid misdiagnosis, unnecessary procedures, or prolonged inappropriate therapy. Prompt recognition and anti-inflammatory treatment can be rapidly effective and permit safe reintroduction of disease-modifying therapy.
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.
Case Report: Switching secukinumab to bimekizumab in diffuse cutaneous systemic sclerosis after autologous hematopoietic stem cell transplantation: a case follow-up.
Diffuse Cutaneous Systemic Sclerosis Complicated by Serum Amyloid A Protein Cardiac Amyloidosis and Cardiogenic Shock.
Efficacy and Safety of Tofacitinib Compared to Cyclophosphamide in Early Diffuse Cutaneous Systemic Sclerosis: A Randomized Controlled Trial.
A retrospective analysis of clinical characteristics of systemic sclerosis-associated interstitial lung disease.
📚 EuropePMC195 artigos no totalmostrando 197
Diffuse Cutaneous Systemic Sclerosis Complicated by Serum Amyloid A Protein Cardiac Amyloidosis and Cardiogenic Shock.
CJC openEfficacy and Safety of Tofacitinib Compared to Cyclophosphamide in Early Diffuse Cutaneous Systemic Sclerosis: A Randomized Controlled Trial.
CureusA retrospective analysis of clinical characteristics of systemic sclerosis-associated interstitial lung disease.
MedicineCrowned Dens Syndrome Mimicking Atlantoaxial Infection in a Patient With Systemic Sclerosis: A Case Report.
Case reports in rheumatologyA randomised open label pilot trial comparing mycophenolate mofetil with no immunosuppression in limited cutaneous systemic sclerosis (MINIMISE-Pilot).
Rheumatology (Oxford, England)Cytometry-based blood immune cell markers associated with clinical outcomes in systemic sclerosis: protocol for a systematic review.
BMJ openEngaging the PD-1 pathway in systemic sclerosis attenuates inflammation-driven fibrosis.
Annals of the rheumatic diseasesLymphocyte Activation Gene 3 Regulation of Profibrotic Cytokines and Type I Collagen Production in Patients With Systemic Sclerosis.
ACR open rheumatologyIdentification of novel fibroblast subsets in diffuse cutaneous systemic sclerosis.
Annals of the rheumatic diseasesBaricitinib ameliorates skin fibrosis via direct fibroblast suppression and endothelial exosome-mediated paracrine signaling.
Arthritis research & therapyMultimodal analyses of early, untreated systemic sclerosis skin identify a proinflammatory vascular niche of macrophage-fibroblast signaling.
JCI insightAssessment of alveolar capillary membrane permeability using technetium 99m-diethylene triamine pentaacetate (Tc99m-DTPA) aerosols in patients with diffuse cutaneous systemic sclerosis - a cross-sectional study.
Expert review of medical devicesA composite biomarker score to predict modified Rodnan skin score in systemic sclerosis: insight from autologous stem cell transplantation international scleroderma trial.
Arthritis research & therapyA pharmacokinetics study to evaluate drug-drug interactions between fipaxalparant and concomitant medications in healthy participants.
Clinical pharmacology in drug developmentFetal and maternal outcome in the pregnancies of patients with systemic sclerosis and very early diagnosis of systemic sclerosis in France: a prospective study.
The Lancet. RheumatologyAn Unusual Case of Seronegative Systemic Sclerosis and Cardiac Tamponade.
CureusSkin Biopsies Enhance Prediction of Clinical Trajectory in Diffuse Cutaneous Systemic Sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)The use of JAK-1 selective inhibitor filgotinib to manage a rare case of concurrent cervical spine calcinosis and inflammatory changes of the odontoid process in diffuse cutaneous systemic sclerosis.
Clinical and experimental rheumatologyCirculating adiponectin levels in systemic sclerosis: A meta-analysis and bidirectional Mendelian randomization study.
Journal of scleroderma and related disordersDifferences in nailfold capillaroscopy findings between limited and diffuse cutaneous systemic sclerosis: a detailed analysis.
RMD openAn iPSC-derived CD19/BCMA CAR-NK therapy in a patient with systemic sclerosis.
CellUpadacitinib as a potential management option for diffuse cutaneous systemic sclerosis: A case report.
SAGE open medical case reportsPatient-reported gastrointestinal involvement is associated with reduced quality of life and disability in systemic sclerosis.
Journal of scleroderma and related disordersCognitive debriefing of the Scleroderma Health Assessment Questionnaire in diffuse cutaneous systemic sclerosis.
Journal of scleroderma and related disordersExamining the relationship between vascular biomarkers and both microangiopathy and cutaneous fibrosis in systemic sclerosis.
Journal of scleroderma and related disordersAutologous hematopoietic stem-cell transplantation in diffuse cutaneous systemic sclerosis: a single-center experience.
ReumatismoFrequency of Disease Subsets and Spectrum of Organ Involvement and Interstitial Lung Disease Patterns in Patients of Systemic Sclerosis in Pakistani Population.
Journal of the College of Physicians and Surgeons--Pakistan : JCPSPBilateral Keratoconus in Diffuse Cutaneous Systemic Sclerosis: A Rare Presentation - Is There Any Role of Autoimmunity?
Turkish journal of ophthalmologyGastroesophageal Intussusception Treated With Combined Transoral Incisionless Fundoplication in a Patient With Systemic Sclerosis.
ACG case reports journalSerum type I interferon score as a disease activity biomarker in patients with diffuse cutaneous systemic sclerosis: a retrospective cohort study.
The Lancet. RheumatologyThe minimal clinically important difference of the scleroderma clinical trials consortium damage index.
Journal of scleroderma and related disorders[Diffuse hyperpigmentation in systemic sclerosis].
Dermatologie (Heidelberg, Germany)Is there a link between choroidal and retinal parameters in patients with systemic sclerosis? A prospective study.
Photodiagnosis and photodynamic therapyBelumosudil in diffuse cutaneous systemic sclerosis: a randomized, double-blind, open-label extension, placebo-controlled, phase 2 study.
Rheumatology (Oxford, England)Diffuse Cutaneous Systemic Sclerosis With Normotensive Scleroderma Renal Crisis and Myopericarditis: A Case Report.
CureusComparative Efficacy of Immunosuppressive Therapies in the Treatment of Diffuse Cutaneous Systemic Sclerosis.
ACR open rheumatologyImprovement in long-term survival with mesenchymal stem cell transplantation in systemic sclerosis patients: a propensity score-matched cohort study.
Stem cell research & therapySystemic sclerosis complicated by azathioprine-induced iatrogenic immunodeficiency-associated lymphoproliferative disorder: A case report.
Modern rheumatology case reportsArrhythmia as a Possible Complication of Mycophenolate Mofetil in Systemic Sclerosis: A Case Report.
Case reports in medicineManagement of disease progression after autologous hematopoietic stem cell transplantation in systemic sclerosis: Results from an international questionnaire-based study.
Seminars in arthritis and rheumatismA phase 2 randomized trial of safety and pharmacokinetics of IgPro20 and IgPro10 in patients with diffuse cutaneous systemic sclerosis.
Rheumatology (Oxford, England)Dysfunctional KLRB1+CD8+ T-cell responses are generated in chronically inflamed systemic sclerosis skin.
Annals of the rheumatic diseasesPsychosocial moderators of the association between pain intensity and physical function in people with systemic sclerosis.
Journal of scleroderma and related disordersSystemic sclerosis-associated severe gastric antral vascular ectasia treated with tocilizumab:A case report and review of the literature.
Journal of scleroderma and related disordersQuantitative 18F-FDG PET-CT can assess presence and extent of interstitial lung disease in early severe diffuse cutaneous systemic sclerosis.
Arthritis research & therapyHospitalizations in patients with systemic sclerosis: Differences between limited and diffuse cutaneous subtypes.
Journal of scleroderma and related disordersPhenotype of diffuse cutaneous systemic sclerosis patients with positive anticentromere antibodies: A systematic literature review and meta-analysis.
Seminars in arthritis and rheumatismAutologous Nonmyeloablative Hematopoietic Stem Cell Transplantation for Diffuse Cutaneous Systemic Sclerosis: Identifying Disease Risk Factors for Toxicity and Long-Term Outcomes in a Prospective, Single-Arm Trial.
Arthritis & rheumatology (Hoboken, N.J.)Design of CONQUEST, a novel, randomized, placebo-controlled, Phase 2b platform clinical trial to investigate new treatments for patients with early active systemic sclerosis with interstitial lung disease.
Journal of scleroderma and related disordersOral Glucocorticoids for Skin Fibrosis in Early Diffuse Systemic Sclerosis: A Target Trial Emulation Study From the European Scleroderma Trials and Research Group Database.
Arthritis care & researchPrevalence, causes, and clinical associations of anemia in patients with systemic sclerosis: A cohort study.
Journal of scleroderma and related disordersDoes regression of skin thickening predict improvement of internal organ involvement and survival in patients with diffuse cutaneous systemic sclerosis? A EUSTAR analysis.
Arthritis research & therapyLong-term outcome of autologous haematopoietic stem cell transplantation in patients with systemic sclerosis: a comparison with patients treated with rituximab and with traditional immunosuppressive agents.
Arthritis research & therapyKidney Transplantation in a Patient with Scleroderma.
The Israel Medical Association journal : IMAJAutologous hematopoietic stem cell transplant for systemic sclerosis associated interstitial lung disease.
Current opinion in rheumatologyDisturbed Complement Receptor Expression Pattern of B Cells Is Enhanced by Toll-like Receptor CD180 Ligation in Diffuse Cutaneous Systemic Sclerosis.
International journal of molecular sciencesSuperior mesenteric artery syndrome - An unusual presentation associated with diffuse cutaneous systemic sclerosis in two cases.
Indian journal of dermatology, venereology and leprologyToll-like Receptor Homologue CD180 Ligation of B Cells Upregulates Type I IFN Signature in Diffuse Cutaneous Systemic Sclerosis.
International journal of molecular sciencesAllogeneic CD19-targeted CAR-T therapy in patients with severe myositis and systemic sclerosis.
CellFamilial aggregation of diffuse cutaneous systemic sclerosis: Interplay of C1r gene defect, susceptible HLA haplotype and autoantibodies.
International journal of rheumatic diseasesAssessing hand and global disability in a cohort of Algerian patients with systemic sclerosis: Construct validities of the Arab Hand Function Index and the Arabic Health Assessment Questionnaire.
Journal of scleroderma and related disordersTreatment Outcomes of Scleroderma With Janus Kinase Inhibitors: A Systematic Review.
Journal of cutaneous medicine and surgeryInterferon-γ Induces Interleukin-6 Production and Alpha-smooth Muscle Actin Expression in Systemic Sclerosis Fibroblasts.
Iranian journal of allergy, asthma, and immunologyNuclear-penetrating scleroderma autoantibody inhibits topoisomerase 1 cleavage complex formation.
Biochemical and biophysical research communicationsPharmacokinetics of Fipaxalparant, a Small-Molecule Selective Negative Allosteric Modulator of Lysophosphatidic Acid Receptor 1, and the Effect of Food in Healthy Volunteers.
Clinical pharmacology in drug developmentEffect of Hepatic and Renal Impairment on the Pharmacokinetics of Dersimelagon (MT-7117), an Oral Melanocortin-1 Receptor Agonist.
Clinical pharmacology in drug developmentBrentuximab vedotin for skin involvement in refractory diffuse cutaneous systemic sclerosis, an open-label trial.
Rheumatology (Oxford, England)Outcomes of Patients With Diffuse Systemic Sclerosis Eligible for Autologous Stem Cell Transplantation Treated With Conventional Therapy.
Arthritis & rheumatology (Hoboken, N.J.)Immune Checkpoint Inhibitor-Associated Systemic Sclerosis in the Treatment of a Small Cell Lung Cancer Patient with Durvalumab: A Case Report.
Clinical, cosmetic and investigational dermatologyBiomarker analysis from the phase 2b randomized placebo-controlled trial of riociguat in early diffuse cutaneous systemic sclerosis.
Rheumatology (Oxford, England)Associations of Digital Ulcers in Patients with Systemic Sclerosis: An 8-Year Retrospective Study.
Dermatology (Basel, Switzerland)Acute Respiratory Distress Syndrome in a Patient With Systemic Sclerosis: A Case of a Life-Threatening Complication.
CureusExploratory clinical subgroup clustering in systemic sclerosis: Results from the Indian Progressive Systemic Sclerosis Registry.
Journal of scleroderma and related disordersRiociguat in patients with early diffuse cutaneous systemic sclerosis (RISE-SSc): open-label, long-term extension of a phase 2b, randomised, placebo-controlled trial.
The Lancet. RheumatologyCase report of anti-survival motor neuron complex antibody-positive overlap syndrome of diffuse cutaneous systemic sclerosis and idiopathic inflammatory myopathies.
The Journal of dermatologyMacular choroidal thickness, volume, and vascularity index in patients with systemic sclerosis.
Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie[Expression and clinical significance of plasma exosomal miR-34-5p and miR-142-3p in systemic sclerosis].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesRapid Progression of Skin Sclerosis Following Surgery for Carpal Tunnel Syndrome: A Case of Diffuse Cutaneous Systemic Sclerosis.
CureusKidney Transplant Outcomes in Patients With Scleroderma an Experience at Northwestern Memorial Hospital, Chicago, Illinois.
Transplantation proceedingsPeripapillary choroidal vascularity index and thickness in patients with systemic sclerosis.
Frontiers in medicineScleroderma renal crisis triggered by ibuprofen: Insights on complement-directed therapy.
Journal of scleroderma and related disordersAnti-thymocyte globulin exposure in patients with diffuse cutaneous systemic sclerosis undergoing autologous haematopoietic stem cell transplantation.
Journal of scleroderma and related disordersReference value for the 6-min walking distance in women with systemic sclerosis considering the impact of muscle strength.
Clinical biomechanics (Bristol, Avon)Metabolic fingerprinting of systemic sclerosis: a systematic review.
Frontiers in molecular biosciencesSingle-cell analysis reveals key differences between early-stage and late-stage systemic sclerosis skin across autoantibody subgroups.
Annals of the rheumatic diseasesCutaneous Features, Autoantibody Profile, and Nailfold Capillaroscopy of Systemic Sclerosis: A Study of 60 Cases.
The Journal of the Association of Physicians of IndiaA rare case of signet ring cell carcinoma with diffuse cutaneous systemic sclerosis: A case report.
The journal of obstetrics and gynaecology researchSubcutaneous immunoglobulin therapy for refractory skin thickening in rapidly progressive systemic sclerosis: A case report and literature review.
Journal of scleroderma and related disordersComprehensive description of the prevalence, serological and clinical characteristics, and visceral involvement of systemic sclerosis (scleroderma) in a large cohort from the United Arab Emirates Systemic Sclerosis Registry.
Journal of scleroderma and related disordersCopeptin as a Biomarker of Microcirculation Alterations in Systemic Sclerosis.
Clinical, cosmetic and investigational dermatologyEfficacy and Safety of Lenabasum, a Cannabinoid Type 2 Receptor Agonist, in a Phase 3 Randomized Trial in Diffuse Cutaneous Systemic Sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)Absorption, metabolism, and excretion of [14 C]dersimelagon, an investigational oral selective melanocortin 1 receptor agonist, in preclinical species and healthy volunteers.
Pharmacology research & perspectivesBilateral Optic Neuropathy in Association with Diffuse Cutaneous Systemic Sclerosis with Interstitial Lung Disease.
Ocular immunology and inflammationEfficacy of Fractional CO2 Laser for Improvement of Limited Mouth Opening in Systemic Sclerosis.
Journal of cutaneous and aesthetic surgerySalivary Flow Rate and Oral Status in Patients with Primary Sjögren's Syndrome and Diffuse Cutaneous Systemic Sclerosis: A Cross-Sectional Study.
Diagnostics (Basel, Switzerland)Purtscher-like Retinopathy in a Patient with Systemic Sclerosis: A Case Report and Narrative Review.
BiomedicinesNative T1 mapping in early diffuse and limited systemic sclerosis, and its association with diastolic function.
Journal of cardiologyBrachial artery flow-mediated dilation in patients with systemic sclerosis: an experience from tertiary care center from North India.
Clinical rheumatologyThe Oral Bioavailability and Effect of Various Gastric Conditions on the Pharmacokinetics of Dersimelagon in Healthy Adult Volunteers.
Clinical pharmacology in drug developmentImpact of Innovative Treatment Using Biological Drugs for the Modulation of Diffuse Cutaneous Systemic Sclerosis: A Systematic Review.
Medicina (Kaunas, Lithuania)A 24-Week, Phase IIa, Randomized, Double-Blind, Placebo-Controlled Study of Ziritaxestat in Early Diffuse Cutaneous Systemic Sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)Prevalence of and factors independently associated with digital ischemic complications in patients with systemic sclerosis.
Journal of scleroderma and related disordersGiant cell arteritis with myeloperoxidase anti-neutrophil cytoplasmic antibody seropositivity in a patient with systemic sclerosis.
Modern rheumatology case reportsCase of systemic sclerosis overlapping with psoriasis.
International journal of rheumatic diseasesEvaluation of choroidal vascularity index in systemic sclerosis patients.
Photodiagnosis and photodynamic therapySilica associated systemic sclerosis: an occupational health hazard.
BMJ case reportsA Phase II randomized controlled trial of oral prednisolone in early diffuse cutaneous systemic sclerosis (PRedSS).
Rheumatology (Oxford, England)Comparing clinical characteristics of systemic sclerosis with or without interstitial lung disease: A cross-sectional study from a single center of the Chinese Rheumatism Data Center.
Frontiers in medicineTrigeminal Neuralgia as an Initial Presentation of Systemic Autoimmune Diseases: A Case Series.
Mediterranean journal of rheumatologyEpiregulin is a dendritic cell-derived EGFR ligand that maintains skin and lung fibrosis.
Science immunologyThe Relationship between Smoking, Raynaud's Phenomenon, Digital Ulcers, and Skin Thickness in the Waikato Systemic Sclerosis Cohort.
Rheumatology and immunology researchIntegrated analysis of dermal blister fluid proteomics and genome-wide skin gene expression in systemic sclerosis: an observational study.
The Lancet. RheumatologyAgreement Between Physician Evaluation and the Composite Response Index in Diffuse Cutaneous Systemic Sclerosis.
Arthritis care & researchThree Distinct Transcriptional Profiles of Monocytes Associate with Disease Activity in Scleroderma Patients.
Arthritis & rheumatology (Hoboken, N.J.)Myeloablative autologous haematopoietic stem cell transplantation resets the B cell repertoire to a more naïve state in patients with systemic sclerosis.
Annals of the rheumatic diseasesSex-specific risk of anti-topoisomerase antibodies on mortality and disease severity in systemic sclerosis: 10-year analysis of the Leiden CCISS and EUSTAR cohorts.
The Lancet. RheumatologyDirect and indirect health-related costs of systemic sclerosis in New Zealand.
International journal of rheumatic diseasesSystemic Sclerosis, Malnutrition, and Small Bowel Obstruction: Why Clinicians Should Consider Early Total Parenteral Nutrition in Systemic Sclerosis With Severe Gastrointestinal Involvement.
CureusJAK1/2 Inhibitor Baricitinib Improves Skin Fibrosis and Digital Ulcers in Systemic Sclerosis.
Frontiers in medicineRevisiting the disease specificity and nomenclature of ficolin-1-positive monocyte-derived dendritic cells in diffuse cutaneous systemic sclerosis: comment on the article by Xue et al.
Arthritis & rheumatology (Hoboken, N.J.)Autoantibodies versus Skin Fibrosis Extent in Systemic Sclerosis: A Case-Control Study of Inverted Phenotypes.
Diagnostics (Basel, Switzerland)Systemic sclerosis manifestations and clinical outcomes in Hispanics/Latinos of the American Southwest.
Journal of scleroderma and related disordersOrthogonal quantification of soluble inducible T-cell costimulator (ICOS) in healthy and diseased human serum.
Journal of pharmaceutical analysisGenome-wide DNA methylation pattern in systemic sclerosis microvascular endothelial cells: Identification of epigenetically affected key genes and pathways.
Journal of scleroderma and related disordersImmunosuppression use in early systemic sclerosis may be increasing over time.
Journal of scleroderma and related disordersHigh D-dimer plasma concentration in systemic sclerosis patients: Prevalence and association with vascular complications.
Journal of scleroderma and related disordersInformation preferences about treatment options in diffuse cutaneous systemic sclerosis: A Delphi consensus study.
Journal of scleroderma and related disordersBelumosudil with ROCK-2 inhibition: chemical and therapeutic development to FDA approval for the treatment of chronic graft-versus-host disease.
Current research in translational medicineAntinuclear antibody pattern and autoantibody profiling of systemic sclerosis patients in a tertiary referral center in North India.
Pathology internationalSkin involvement in early diffuse cutaneous systemic sclerosis: an unmet clinical need.
Nature reviews. RheumatologyChronic dyspnea with Raynaud's phenomenon and elevated ANA: A diagnosis of systemic sclerosis sine scleroderma.
The American journal of the medical sciencesClinical worsening following discontinuation of tocilizumab in diffuse cutaneous systemic sclerosis: a single-centre experience in Japan.
Rheumatology (Oxford, England)Diffuse cutaneous systemic sclerosis following SARS-Co V-2 vaccination.
Journal of autoimmunityCannabinoids for the Treatment of Dermatologic Conditions.
JID innovations : skin science from molecules to population healthAssociations between the Composite Response Index in Diffuse Cutaneous Systemic Sclerosis (CRISS), survival and other disease measures.
Seminars in arthritis and rheumatismSpontaneous pneumomediastinum developed after steroid pulse therapy in diffuse cutaneous systemic sclerosis patient: A case report.
The Journal of dermatologySafety and efficacy of abatacept in early diffuse cutaneous systemic sclerosis (ASSET): open-label extension of a phase 2, double-blind randomised trial.
The Lancet. RheumatologyClinical features and prognostic factors of systemic sclerosis in Guangxi, China: Retrospective, single-center study of long-term survival in 470 patients.
International journal of rheumatic diseasesManagement of pregnancy with diffuse cutaneous systemic sclerosis: a case report and literature review.
The Journal of international medical researchAnti-polymyositis/Scl antibody-positive overlap syndrome of diffuse cutaneous systemic sclerosis, dermatomyositis, systemic lupus erythematosus, and antiphospholipid syndrome.
The Journal of dermatologyOcclusive retinal vasculitis associated with systemic sclerosis and antiphospholipid antibodies.
American journal of ophthalmology case reportsTherapeutic Approaches to Systemic Sclerosis: Recent Approvals and Future Candidate Therapies.
Clinical reviews in allergy & immunologyAutologous stem-cell transplantation in systemic sclerosis-associated interstitial lung disease: early action in selected patients rather than escalation therapy for all.
Therapeutic advances in musculoskeletal diseaseA randomised, double-blind, placebo-controlled phase 3 study of lenabasum in diffuse cutaneous systemic sclerosis: RESOLVE-1 design and rationale.
Clinical and experimental rheumatologyOutcomes linked to eligibility for stem cell transplantation trials in diffuse cutaneous systemic sclerosis.
Rheumatology (Oxford, England)Increased Frequency of Activated Switched Memory B Cells and Its Association With the Presence of Pulmonary Fibrosis in Diffuse Cutaneous Systemic Sclerosis Patients.
Frontiers in immunologySuccessful treatment with baricitinib of refractory arthritis in a patient with severe diffuse cutaneous systemic sclerosis-rheumatoid arthritis overlap syndrome.
Clinical and experimental rheumatologyMolecular basis for clinical diversity between autoantibody subsets in diffuse cutaneous systemic sclerosis.
Annals of the rheumatic diseasesClinical trial protocol: PRednisolone in early diffuse cutaneous Systemic Sclerosis (PRedSS).
Journal of scleroderma and related disordersPain and Self-Efficacy Among Patients With Systemic Sclerosis: A Scleroderma Patient-Centered Intervention Network Cohort Study.
Nursing researchClinical characteristics, visceral involvement, and mortality in at-risk or early diffuse systemic sclerosis: a longitudinal analysis of an observational prospective multicenter US cohort.
Arthritis research & therapyComprehensive analysis of the major histocompatibility complex in systemic sclerosis identifies differential HLA associations by clinical and serological subtypes.
Annals of the rheumatic diseasesExpansion of Fcγ Receptor IIIa-Positive Macrophages, Ficolin 1-Positive Monocyte-Derived Dendritic Cells, and Plasmacytoid Dendritic Cells Associated With Severe Skin Disease in Systemic Sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)The Efficacy of a Home-Based, Self-Administered Hand Exercise Program for Patients With Systemic Sclerosis: A Randomized Controlled, Evaluator-Blind, Clinical Trial.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesAnti-tRNA synthetase syndrome interstitial lung disease: A single center experience.
Respiratory medicineCirculating Galectin-1 and Galectin-3 in Sera From Patients With Systemic Sclerosis: Associations With Clinical Features and Treatment.
Frontiers in pharmacologyIntensive and app-delivered occupational therapy to improve upper extremity function in early diffuse cutaneous systemic sclerosis: a pilot two-arm trial.
Rheumatology (Oxford, England)Analysis of PI3K Pathway Associated Molecules Reveals Dysregulated Innate and Adaptive Functions of B Cells in Early Diffuse Cutaneous Systemic Sclerosis.
International journal of molecular sciencesSystemic Sclerosis and Pulmonary Disease.
Advances in experimental medicine and biologyIncreased malignancies in our Waikato cohort of patients with systemic sclerosis.
International journal of rheumatic diseasesA randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: the UPSIDE study protocol.
BMJ openDevelopment of Two Types of Skin Cancer in a Patient with Systemic Sclerosis: a Case Report and Overview of the Literature.
Case reports in oncological medicineDevelopment of a Musculoskeletal Ultrasound Protocol to Examine Upper Extremity Rehabilitation Outcomes in Systemic Sclerosis.
Journal of diagnostic medical sonography : JDMSDefining the optimal disease duration of early diffuse systemic sclerosis for clinical trial design.
Rheumatology (Oxford, England)Lymphocyte subset abnormalities in early diffuse cutaneous systemic sclerosis.
Arthritis research & therapyVerteporfin inhibits the persistent fibrotic phenotype of lesional scleroderma dermal fibroblasts.
Journal of cell communication and signalingFinger sweating levels evaluated by video capillaroscopy system are increased in patients with systemic sclerosis compared to pre-clinical stage patients.
Drug discoveries & therapeuticsNew composite endpoint in early diffuse cutaneous systemic sclerosis: revisiting the provisional American College of Rheumatology Composite Response Index in Systemic Sclerosis.
Annals of the rheumatic diseasesPrevalence and clinical associations with premature ovarian insufficiency, early menopause, and low ovarian reserve in systemic sclerosis.
Clinical rheumatologyRiociguat: Clinical research and evolving role in therapy.
British journal of clinical pharmacologyLung transplantation resulted in marked improvement of autoimmunity and scleroderma in diffuse cutaneous systemic sclerosis: a case report.
Rheumatology (Oxford, England)Tofacitinib as a possible treatment for skin thickening in diffuse cutaneous systemic sclerosis.
Rheumatology (Oxford, England)Large-Scale Characterization of Systemic Sclerosis Serum Protein Profile: Comparison to Peripheral Blood Cell Transcriptome and Correlations With Skin/Lung Fibrosis.
Arthritis & rheumatology (Hoboken, N.J.)Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database.
Arthritis research & therapyReduced salivary flow and caries status are correlated with disease activity and severity in patients with diffuse cutaneous systemic sclerosis.
The Journal of international medical researchUtilization of Dual-Energy Computed Tomography in Evaluating Calcinosis Cutis in a Patient With Diffuse Cutaneous Systemic Sclerosis.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesMachine learning integration of scleroderma histology and gene expression identifies fibroblast polarisation as a hallmark of clinical severity and improvement.
Annals of the rheumatic diseasesFrom "being at war" to "getting back on your feet": A qualitative study on experiences of patients with systemic sclerosis treated with hematopoietic stem cell transplantation.
Journal of scleroderma and related disordersA randomised, double-blind, placebo-controlled, 24-week, phase II, proof-of-concept study of romilkimab (SAR156597) in early diffuse cutaneous systemic sclerosis.
Annals of the rheumatic diseasesA case of Myhre syndrome mimicking juvenile scleroderma.
Pediatric rheumatology online journalMycobacterium avium complex infection in a patient with systemic sclerosis- associated interstitial lung disease: A case report.
Respiratory medicine case reportsTocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial.
The Lancet. Respiratory medicineRapid skin thickness progression rate is associated with high incidence rate of cardiopulmonary complications in patients with early diffuse cutaneous systemic sclerosis: inception cohort study.
Clinical and experimental rheumatologyPulmonary mucormycosis following autologous hematopoietic stem cell transplantation for rapidly progressive diffuse cutaneous systemic sclerosis: A case report.
MedicineDistinct gene network in skin lesion of patients with diffuse cutaneous systemic sclerosis.
Clinical rheumatologyPerformance Characteristics of Pulmonary Function Tests for the Detection of Interstitial Lung Disease in Adults With Early Diffuse Cutaneous Systemic Sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)Comparison of the disease activity score and the revised EUSTAR activity index in diffuse cutaneous systemic sclerosis patients.
Clinical and experimental rheumatologyInitial predictors of skin thickness progression in patients with diffuse cutaneous systemic sclerosis: Results from a multicentre prospective cohort in Japan.
Modern rheumatologyImmunosuppressive treatment in diffuse cutaneous systemic sclerosis is associated with an improved composite response index (CRISS).
Arthritis research & therapyThe Doubled-Edged Sword of T1-Mapping in Systemic Sclerosis-A Comparison with Infectious Myocarditis Using Cardiovascular Magnetic Resonance.
Diagnostics (Basel, Switzerland)Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment.
Clinical rheumatologyLenabasum for Skin Disease in Patients With Diffuse Cutaneous Systemic Sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)Safety and Efficacy of Lenabasum in a Phase II, Randomized, Placebo-Controlled Trial in Adults With Systemic Sclerosis.
Arthritis & rheumatology (Hoboken, N.J.)Riociguat in patients with early diffuse cutaneous systemic sclerosis (RISE-SSc): randomised, double-blind, placebo-controlled multicentre trial.
Annals of the rheumatic diseasesClinical course of Japanese patients with early systemic sclerosis: A multicenter, prospective, observational study.
Modern rheumatologyNOTCH3 T6746C and TP53 P72R Polymorphisms Are Associated with the Susceptibility to Diffuse Cutaneous Systemic Sclerosis.
BioMed research internationalDrugs in phase I and phase II clinical trials for systemic sclerosis.
Expert opinion on investigational drugsAssociações
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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.
- Cytometry-based blood immune cell markers associated with clinical outcomes in systemic sclerosis: protocol for a systematic review.
- Fetal and maternal outcome in the pregnancies of patients with systemic sclerosis and very early diagnosis of systemic sclerosis in France: a prospective study.
- Efficacy and Safety of Tofacitinib Compared to Cyclophosphamide in Early Diffuse Cutaneous Systemic Sclerosis: A Randomized Controlled Trial.
- A retrospective analysis of clinical characteristics of systemic sclerosis-associated interstitial lung disease.
- Crowned Dens Syndrome Mimicking Atlantoaxial Infection in a Patient With Systemic Sclerosis: A Case Report.
- 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.
- Case Report: Switching secukinumab to bimekizumab in diffuse cutaneous systemic sclerosis after autologous hematopoietic stem cell transplantation: a case follow-up.
- Diffuse Cutaneous Systemic Sclerosis Complicated by Serum Amyloid A Protein Cardiac Amyloidosis and Cardiogenic Shock.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:220393(Orphanet)
- MONDO:0016356(MONDO)
- Esclerose Sistemica(PCDT · Ministério da Saúde)
- GARD:9751(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q18554846(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
