2023
Impaired left-ventricular global longitudinal strain by feature-tracking cardiac MRI predicts mortality in systemic sclerosis
Feher A, Miller E, Peters D, Mojibian H, Sinusas A, Hinchcliff M, Baldassarre L. Impaired left-ventricular global longitudinal strain by feature-tracking cardiac MRI predicts mortality in systemic sclerosis. Rheumatology International 2023, 43: 849-858. PMID: 36894756, DOI: 10.1007/s00296-023-05294-6.Peer-Reviewed Original ResearchConceptsLate gadolinium enhancementLV-GLSSystemic sclerosisWorse survivalClinical indicationsImpaired left ventricular global longitudinal strainLeft ventricular global longitudinal strainPresence of LGECMR strainLV cardiac indexWorse LV-GLSLV ejection fractionCox regression analysisGlobal longitudinal strainLV cardiac outputGlobal radialCardiac indexOverall survivalRetrospective cohortEjection fractionPrognostic valueAdverse outcomesCardiac outputGadolinium enhancementRV strain
2021
Heterogeneity of primary and secondary peristalsis in systemic sclerosis: A new model of “scleroderma esophagus”
Carlson DA, Prescott JE, Germond E, Brenner D, Carns M, Correia CS, Tetreault M, McMahan ZH, Hinchcliff M, Kou W, Kahrilas PJ, Perlman HR, Pandolfino JE. Heterogeneity of primary and secondary peristalsis in systemic sclerosis: A new model of “scleroderma esophagus”. Neurogastroenterology & Motility 2021, 34: e14284. PMID: 34709690, PMCID: PMC9046463, DOI: 10.1111/nmo.14284.Peer-Reviewed Original ResearchConceptsHigh-resolution manometryIneffective esophageal motilitySecondary peristalsisSystemic sclerosisFLIP panometryAbsent contractilityContractile responseDistensibility indexFunctional luminal imaging probe (FLIP) panometryEsophagogastric junction distensibility indexPattern of contractilityEGJ-DIScleroderma esophagusEsophageal dysmotilityReflux severitySSc patientsClinical outcomesEsophageal motilityManometric diagnosisPrimary peristalsisMotor abnormalitiesNormal peristalsisEsophageal functionChicago ClassificationPatientsPredictive Significance of Serum Interferon‐Inducible Protein Score for Response to Treatment in Systemic Sclerosis–Related Interstitial Lung Disease
Assassi S, Li N, Volkmann ER, Mayes MD, Rünger D, Ying J, Roth MD, Hinchcliff M, Khanna D, Frech T, Clements PJ, Furst DE, Goldin J, Bernstein EJ, Castelino FV, Domsic RT, Gordon JK, Hant FN, Shah AA, Shanmugam VK, Steen VD, Elashoff RM, Tashkin DP. Predictive Significance of Serum Interferon‐Inducible Protein Score for Response to Treatment in Systemic Sclerosis–Related Interstitial Lung Disease. Arthritis & Rheumatology 2021, 73: 1005-1013. PMID: 33350170, PMCID: PMC8169525, DOI: 10.1002/art.41627.Peer-Reviewed Original ResearchMeSH KeywordsAdultAgedBeta 2-MicroglobulinChemokine CCL19Chemokine CCL8Chemokine CXCL10Chemokine CXCL9CyclophosphamideFemaleHumansImmunosuppressive AgentsLung Diseases, InterstitialMaleMethotrexateMiddle AgedMycophenolic AcidObservational Studies as TopicPrognosisRandomized Controlled Trials as TopicReceptors, Tumor Necrosis Factor, Type IIScleroderma, SystemicVital CapacityConceptsInterstitial lung diseaseMycophenolate mofetilPredictive significanceCYC armILD courseMMF armSSc-ILDSystemic sclerosisLung diseaseHigh baseline C-reactive protein levelsTumor necrosis factor receptor type IIBaseline C-reactive protein levelsScleroderma Lung Study IIC-reactive protein levelsGood responseVital capacity percentChemotactic protein-2Receptor type IIActive immunosuppressionClinical predictorsCRP levelsObservational cohortProtein scoreActive treatmentTreatment armsLarge‐Scale Characterization of Systemic Sclerosis Serum Protein Profile: Comparison to Peripheral Blood Cell Transcriptome and Correlations With Skin/Lung Fibrosis
Bellocchi C, Ying J, Goldmuntz EA, Keyes‐Elstein L, Varga J, Hinchcliff ME, Lyons MA, McSweeney P, Furst DE, Nash R, Crofford LJ, Welch B, Goldin JG, Pinckney A, Mayes MD, Sullivan KM, Assassi S. Large‐Scale Characterization of Systemic Sclerosis Serum Protein Profile: Comparison to Peripheral Blood Cell Transcriptome and Correlations With Skin/Lung Fibrosis. Arthritis & Rheumatology 2021, 73: 660-670. PMID: 33131208, PMCID: PMC8005427, DOI: 10.1002/art.41570.Peer-Reviewed Original ResearchConceptsDiffuse cutaneous systemic sclerosisInterstitial lung diseaseSerum protein profilesHealthy control subjectsControl subjectsSex-matched healthy control subjectsRodnan skin thickness scoreSevere interstitial lung diseaseCutaneous systemic sclerosisMean disease durationDiffuse cutaneous SScWhole blood gene expression profilingSkin thickness scoreBlood gene expression profilingPeripheral blood cellsCancer antigen 15Epidermal growth factor receptorSoluble epidermal growth factor receptorSerum proteinsGrowth factor receptorDisease activityDisease durationSystemic sclerosisCutaneous SScImmunosuppressive agents
2020
Factors associated with fears due to COVID-19: A Scleroderma Patient-centered Intervention Network (SPIN) COVID-19 cohort study
Wu Y, Kwakkenbos L, Henry R, Carrier M, Gagarine M, Harb S, Bourgeault A, Tao L, Carboni-Jiménez A, Negeri Z, Patten S, Bartlett S, Mouthon L, Varga J, Benedetti A, Thombs B, Advisors F, Fortuné C, Gietzen A, Guillot G, Lewis N, Richard M, Sauvé M, Welling J, Fligelstone K, Gottesman K, Leite C, Pérez E, Investigators S, Baron M, Malcarne V, Mayes M, Nielson W, Riggs R, Assassi S, Ells C, van den Ende C, Frech T, Harel D, Hinchcliff M, Hudson M, Johnson S, Larche M, Nguyen C, Pope J, Rannou F, Reyna T, Schouffoer A, Suarez-Almazor M, Agard C, Albert A, Bernstein E, Berthier S, Bissonnette L, Bruns A, Carreira P, Chaigne B, Chung L, Correia C, Denton C, Domsic R, Dunne J, Dunogue B, Farge-Bancel D, Fortin P, Gordon J, Granel-Rey B, Hatron P, Herrick A, Hoa S, Jones N, de B. Fernandes A, Kafaja S, Khalidi N, Launay D, Manning J, Marie I, Martin M, Mekinian A, Melchor S, Nikpour M, Olagne L, Proudman S, Régent A, Rivière S, Robinson D, Rodriguez E, Roux S, Sobanski V, Steen V, Sutton E, Thorne C, Wilcox P, Ayala M, Carboni-Jiménez A, Gagarine M, Nordlund J, Østbø N, Rice D, Turner K, Culos-Reed N, Dyas L, El-Baalbaki G, Hebblethwaite S, Bustamante L, Duchek D, Ellis K. Factors associated with fears due to COVID-19: A Scleroderma Patient-centered Intervention Network (SPIN) COVID-19 cohort study. Journal Of Psychosomatic Research 2020, 140: 110314. PMID: 33271402, PMCID: PMC7685938, DOI: 10.1016/j.jpsychores.2020.110314.Peer-Reviewed Original ResearchChanges in mental health symptoms from pre-COVID-19 to COVID-19 among participants with systemic sclerosis from four countries: A Scleroderma Patient-centered Intervention Network (SPIN) Cohort study
Thombs B, Kwakkenbos L, Henry R, Carrier M, Patten S, Harb S, Bourgeault A, Tao L, Bartlett S, Mouthon L, Varga J, Benedetti A, Advisors F, Fortuné C, Gietzen A, Guillot G, Lewis N, Richard M, Sauvé M, Welling J, Fligelstone K, Gottesman K, Leite C, Pérez E, Investigators S, Baron M, Malcarne V, Mayes M, Nielson W, Riggs R, Assassi S, Ells C, van den Ende C, Frech T, Harel D, Hinchcliff M, Hudson M, Johnson S, Larche M, Nguyen C, Pope J, Rannou F, Reyna T, Schouffoer A, Suarez-Almazor M, Agard C, Albert A, Bernstein E, Berthier S, Bissonnette L, Bruns A, Carreira P, Chaigne B, Chung L, Correia C, Denton C, Domsic R, Dunne J, Dunogue B, Farge-Bancel D, Fortin P, Gordon J, Granel-Rey B, Hatron P, Herrick A, Hoa S, Jones N, de B. Fernandes A, Kafaja S, Khalidi N, Launay D, Manning J, Marie I, Martin M, Mekinian A, Melchor S, Nikpour M, Olagne L, Proudman S, Régent A, Rivière S, Robinson D, Rodriguez E, Roux S, Sobanski V, Steen V, Sutton E, Thorne C, Wilcox P, Ayala M, Carboni-Jiménez A, Gagarine M, Nordlund J, Østbø N, Rice D, Turner K, Culos-Reed N, Dyas L, El-Baalbaki G, Hebblethwaite S, Bustamante L, Duchek D, Ellis K. Changes in mental health symptoms from pre-COVID-19 to COVID-19 among participants with systemic sclerosis from four countries: A Scleroderma Patient-centered Intervention Network (SPIN) Cohort study. Journal Of Psychosomatic Research 2020, 139: 110262. PMID: 33070043, PMCID: PMC7532799, DOI: 10.1016/j.jpsychores.2020.110262.Peer-Reviewed Original ResearchPerformance Characteristics of Pulmonary Function Tests for the Detection of Interstitial Lung Disease in Adults With Early Diffuse Cutaneous Systemic Sclerosis
Bernstein EJ, Jaafar S, Assassi S, Domsic RT, Frech TM, Gordon JK, Broderick RJ, Hant FN, Hinchcliff ME, Shah AA, Shanmugam VK, Steen VD, Khanna D. Performance Characteristics of Pulmonary Function Tests for the Detection of Interstitial Lung Disease in Adults With Early Diffuse Cutaneous Systemic Sclerosis. Arthritis & Rheumatology 2020, 72: 1892-1896. PMID: 32583956, PMCID: PMC7722107, DOI: 10.1002/art.41415.Peer-Reviewed Original ResearchConceptsDetection of ILDPulmonary function testsInterstitial lung diseaseDiffuse cutaneous SScEarly diffuse cutaneous systemic sclerosisDiffuse cutaneous systemic sclerosisNegative predictive valueCutaneous systemic sclerosisSystemic sclerosisFunction testsLung diseaseDevelopment of ILDDiagnosis of ILDEarly diffuse cutaneous SScRadiographic interstitial lung diseaseInadequate screening toolsEarly systemic sclerosisRetrospective cohort studyCause of deathProspective registrySSc cohortCohort studyVital capacityHRCT imagingHigh riskLenabasum for Skin Disease in Patients With Diffuse Cutaneous Systemic Sclerosis
Hinchcliff M. Lenabasum for Skin Disease in Patients With Diffuse Cutaneous Systemic Sclerosis. Arthritis & Rheumatology 2020, 72: 1237-1240. PMID: 32368869, PMCID: PMC7641910, DOI: 10.1002/art.41302.Peer-Reviewed Original ResearchProfibrotic Activation of Human Macrophages in Systemic Sclerosis
Bhandari R, Ball MS, Martyanov V, Popovich D, Schaafsma E, Han S, ElTanbouly M, Orzechowski NM, Carns M, Arroyo E, Aren K, Hinchcliff M, Whitfield ML, Pioli PA. Profibrotic Activation of Human Macrophages in Systemic Sclerosis. Arthritis & Rheumatology 2020, 72: 1160-1169. PMID: 32134204, PMCID: PMC7329566, DOI: 10.1002/art.41243.Peer-Reviewed Original ResearchMeSH KeywordsAdultAntigens, CDAntigens, Differentiation, MyelomonocyticCell DifferentiationChemokine CCL2Coculture TechniquesFemaleFibroblastsFibrosisHLA-DR AntigensHumansImmunophenotypingInterleukin-6Lectins, C-TypeLeukocytes, MononuclearMacrophage ActivationMacrophagesMaleMannose ReceptorMannose-Binding LectinsMiddle AgedMonocytesPhosphorylationReceptor, Transforming Growth Factor-beta Type IReceptor, Transforming Growth Factor-beta Type IIReceptors, Cell SurfaceRNA, MessengerScleroderma, SystemicSkinSTAT3 Transcription FactorTranscriptomeTransforming Growth Factor betaConceptsPeripheral blood mononuclear cellsSystemic sclerosisSSc patientsBasal conditionsSex-matched healthy controlsSSc fibroblastsSurface markersHealthy donor monocytesBlood mononuclear cellsMediator of fibrosisInflammatory macrophage activationMonocyte-derived macrophagesActivation profilesGrowth factor βFibrotic activationGene expression signaturesDonor monocytesMononuclear cellsProfibrotic activationSkin fibrosisInterleukin-6Healthy controlsSSc skinIndependent cohortMacrophage activationHigh-throughput quantitative histology in systemic sclerosis skin disease using computer vision
Correia C, Mawe S, Lofgren S, Marangoni RG, Lee J, Saber R, Aren K, Cheng M, Teaw S, Hoffmann A, Goldberg I, Cowper SE, Khatri P, Hinchcliff M, Mahoney JM. High-throughput quantitative histology in systemic sclerosis skin disease using computer vision. Arthritis Research & Therapy 2020, 22: 48. PMID: 32171325, PMCID: PMC7071594, DOI: 10.1186/s13075-020-2127-0.Peer-Reviewed Original ResearchConceptsSystemic sclerosisFibrosis scoreQuantitative image featuresBiopsy scorePrimary cohortOutcome measuresSecondary cohortDiagnostic scoreSkin severity scoreSSc clinical trialsRodnan skin scoreLogistic regression modelsControl biopsiesRegression modelsSkin scoreSeverity scoreClinical hallmarkClinical trialsForearm biopsiesSSc skinBiopsy sectionsIndependent cohortPatient basisSSc biopsiesCollagen deposition
2019
Global skin gene expression analysis of early diffuse cutaneous systemic sclerosis shows a prominent innate and adaptive inflammatory profile
Skaug B, Khanna D, Swindell WR, Hinchcliff ME, Frech TM, Steen VD, Hant FN, Gordon JK, Shah AA, Zhu L, Zheng WJ, Browning JL, Barron AMS, Wu M, Visvanathan S, Baum P, Franks JM, Whitfield ML, Shanmugam VK, Domsic RT, Castelino FV, Bernstein EJ, Wareing N, Lyons MA, Ying J, Charles J, Mayes MD, Assassi S. Global skin gene expression analysis of early diffuse cutaneous systemic sclerosis shows a prominent innate and adaptive inflammatory profile. Annals Of The Rheumatic Diseases 2019, 79: annrheumdis-2019-215894. PMID: 31767698, PMCID: PMC7386329, DOI: 10.1136/annrheumdis-2019-215894.Peer-Reviewed Original ResearchConceptsImmune cell signaturesEarly diffuse systemic sclerosisDiffuse systemic sclerosisLonger disease durationSystemic sclerosisDisease durationCell signatureSSc patientsT cellsEarly diffuse cutaneous systemic sclerosisScleroderma Outcome Study cohortDiffuse cutaneous systemic sclerosisShorter disease durationCutaneous systemic sclerosisRodnan skin scoreCD8 T cellsB cell signaturesCD4 T cellsSystemic sclerosis cohortImmune cell markersClinical trial designDiffuse SSc patientsBiopsy RNAProspective registryClinical courseMyeloablation followed by autologous stem cell transplantation normalises systemic sclerosis molecular signatures
Assassi S, Wang X, Chen G, Goldmuntz E, Keyes-Elstein L, Ying J, Wallace PK, Turner J, Zheng WJ, Pascual V, Varga J, Hinchcliff ME, Bellocchi C, McSweeney P, Furst DE, Nash RA, Crofford LJ, Welch B, Pinckney A, Mayes MD, Sullivan KM. Myeloablation followed by autologous stem cell transplantation normalises systemic sclerosis molecular signatures. Annals Of The Rheumatic Diseases 2019, 78: 1371-1378. PMID: 31391177, PMCID: PMC7167108, DOI: 10.1136/annrheumdis-2019-215770.Peer-Reviewed Original ResearchMeSH KeywordsAdultCyclophosphamideDown-RegulationFemaleHematopoietic Stem Cell TransplantationHumansInterferonsMaleMiddle AgedMultilevel AnalysisMyeloablative AgonistsNeutrophilsRandomized Controlled Trials as TopicScleroderma, SystemicTranscriptomeTransplantation ConditioningTransplantation, AutologousTreatment OutcomeUp-RegulationConceptsHaematopoietic stem cell transplantationStem cell transplantationSystemic sclerosisNeutrophil modulesCell transplantationAutologous stem cell transplantationMolecular signaturesImproved clinical outcomesSerum protein levelsDisease-related molecular signaturesCYC armMonths postrandomisationBaseline visitSkin scoreClinical outcomesCyclophosphamide treatmentVital capacityPretreatment baselineSignificant changesControl armBlood transcriptsWhole blood transcriptsConventional treatmentInterferonBaseline samplesIncreased monocyte count as a cellular biomarker for poor outcomes in fibrotic diseases: a retrospective, multicentre cohort study
Scott MKD, Quinn K, Li Q, Carroll R, Warsinske H, Vallania F, Chen S, Carns MA, Aren K, Sun J, Koloms K, Lee J, Baral J, Kropski J, Zhao H, Herzog E, Martinez FJ, Moore BB, Hinchcliff M, Denny J, Kaminski N, Herazo-Maya JD, Shah NH, Khatri P. Increased monocyte count as a cellular biomarker for poor outcomes in fibrotic diseases: a retrospective, multicentre cohort study. The Lancet Respiratory Medicine 2019, 7: 497-508. PMID: 30935881, PMCID: PMC6529612, DOI: 10.1016/s2213-2600(18)30508-3.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisPulmonary fibrosisAbsolute monocyte countMonocyte countImmune cell typesElectronic health recordsPoor outcomeHigh riskSystemic sclerosisMonocyte percentageHypertrophic cardiomyopathyHigh absolute monocyte countPeripheral blood mononuclear cell samplesComplete blood count valuesSpecific immune cell typesTransplant-free survivalMulticentre cohort studyHealth recordsHigh-risk patientsBlood count valuesSame clinical presentationHigher monocyte countMononuclear cell samplesRisk of mortalityCell types
2018
Performance of Forced Vital Capacity and Lung Diffusion Cutpoints for Associated Radiographic Interstitial Lung Disease in Systemic Sclerosis
Showalter K, Hoffmann A, Rouleau G, Aaby D, Lee J, Richardson C, Dematte J, Agrawal R, Chang RW, Hinchcliff M. Performance of Forced Vital Capacity and Lung Diffusion Cutpoints for Associated Radiographic Interstitial Lung Disease in Systemic Sclerosis. The Journal Of Rheumatology 2018, 45: 1572-1576. PMID: 30275265, PMCID: PMC6214765, DOI: 10.3899/jrheum.171362.Peer-Reviewed Original ResearchConceptsScl-70 autoantibodiesPulmonary function testsNegative predictive valueInterstitial lung diseaseSSc-ILDVital capacityLung diseaseSystemic sclerosis-associated interstitial lung diseaseRadiographic interstitial lung diseaseHigh negative predictive valueReceiver-operating characteristic curveForced Vital CapacityILD evaluationChest HRCTSystemic sclerosisFunction testsTomography scanAmerican CollegeFVCDLCOPatientsAutoantibodiesPredictive valueSSc criteriaThoracic radiologistsNormal Values of Esophageal Distensibility and Distension-Induced Contractility Measured by Functional Luminal Imaging Probe Panometry
Carlson DA, Kou W, Lin Z, Hinchcliff M, Thakrar A, Falmagne S, Prescott J, Dorian E, Kahrilas PJ, Pandolfino JE. Normal Values of Esophageal Distensibility and Distension-Induced Contractility Measured by Functional Luminal Imaging Probe Panometry. Clinical Gastroenterology And Hepatology 2018, 17: 674-681.e1. PMID: 30081222, PMCID: PMC6360138, DOI: 10.1016/j.cgh.2018.07.042.Peer-Reviewed Original ResearchConceptsRepetitive antegrade contractionsEGJ-DIFLIP panometryDistensibility plateauAntegrade contractionsEsophageal contractilityEsophagogastric junctionEsophageal distensibilityAsymptomatic volunteersClinical practiceFunctional luminal imaging probe (FLIP) panometryEGJ-distensibility indexSedated upper endoscopyTime of endoscopyProximal esophageal bodyRAC patternUpper endoscopyDistal esophagusEosinophilic esophagitisEsophageal bodyNormal findingsProspective studyEsophageal diseaseDistal bodyHealthy individualsMycophenolate Mofetil Treatment of Systemic Sclerosis Reduces Myeloid Cell Numbers and Attenuates the Inflammatory Gene Signature in Skin
Hinchcliff M, Toledo DM, Taroni JN, Wood TA, Franks JM, Ball MS, Hoffmann A, Amin SM, Tan AU, Tom K, Nesbeth Y, Lee J, Ma M, Aren K, Carns MA, Pioli PA, Whitfield ML. Mycophenolate Mofetil Treatment of Systemic Sclerosis Reduces Myeloid Cell Numbers and Attenuates the Inflammatory Gene Signature in Skin. Journal Of Investigative Dermatology 2018, 138: 1301-1310. PMID: 29391252, PMCID: PMC6590516, DOI: 10.1016/j.jid.2018.01.006.Peer-Reviewed Original ResearchConceptsMycophenolate mofetil treatmentMyeloid cell numbersMMF therapyMofetil treatmentSystemic sclerosisInflammatory scoreSkin biopsiesCell numberSkin myeloid cellsMyeloid dendritic cellsHalf of patientsRodnan skin scoreImmune cell numbersInflammatory gene signatureExpression of chemokinesProtein levelsCCL2 protein levelsCCL2 mRNA expressionInflammatory signatureDendritic cellsSkin scoreCCL2 mRNAEleven subjectsMonocyte migrationMyeloid cells
2017
Brief Report: Association of Elevated Adipsin Levels With Pulmonary Arterial Hypertension in Systemic Sclerosis
Korman BD, Marangoni RG, Hinchcliff M, Shah SJ, Carns M, Hoffmann A, Ramsey‐Goldman R, Varga J. Brief Report: Association of Elevated Adipsin Levels With Pulmonary Arterial Hypertension in Systemic Sclerosis. Arthritis & Rheumatology 2017, 69: 2062-2068. PMID: 28651038, PMCID: PMC5748338, DOI: 10.1002/art.40193.Peer-Reviewed Original ResearchMeSH KeywordsAdiponectinAdultAgedAutoantibodiesComplement Factor DCytokinesFemaleGene Expression ProfilingHumansHypertension, PulmonaryLeptinMaleMiddle AgedNatriuretic Peptide, BrainNicotinamide PhosphoribosyltransferaseOdds RatioPolymorphism, Single NucleotideResistinScleroderma, DiffuseScleroderma, LimitedConceptsSSc-related pulmonary arterial hypertensionPulmonary arterial hypertensionSystemic sclerosisAdipokine levelsComplement pathway activationAdipsin levelsArterial hypertensionLimited cutaneous systemic sclerosisB-type natriuretic peptidePathway activationSerum adipokine levelsCutaneous systemic sclerosisLevels of adiponectinGene single nucleotide polymorphismsAdipose tissue biologyTissue-derived markersAdipokine balanceAutoantibody statusPulmonary functionSSc patientsAdipocyte dysfunctionClinical featuresPathogenic linkClinical manifestationsNatriuretic peptideReliability and Validity of the Tender and Swollen Joint Counts and the Modified Rodnan Skin Score in Early Diffuse Cutaneous Systemic Sclerosis: Analysis from the Prospective Registry of Early Systemic Sclerosis Cohort.
Gordon JK, Girish G, Berrocal VJ, Zhang M, Hatzis C, Assassi S, Bernstein EJ, Domsic RT, Hant FN, Hinchcliff M, Schiopu E, Steen VD, Frech TM, Khanna D. Reliability and Validity of the Tender and Swollen Joint Counts and the Modified Rodnan Skin Score in Early Diffuse Cutaneous Systemic Sclerosis: Analysis from the Prospective Registry of Early Systemic Sclerosis Cohort. The Journal Of Rheumatology 2017, 44: 791-794. PMID: 28298560, PMCID: PMC5457319, DOI: 10.3899/jrheum.160654.Peer-Reviewed Original ResearchConceptsDiffuse cutaneous systemic sclerosisSwollen joint countCutaneous systemic sclerosisRodnan skin scoreMusculoskeletal ultrasoundJoint countSkin scoreSystemic sclerosisEarly diffuse cutaneous systemic sclerosisModified Rodnan skin scoreSystemic sclerosis cohortProspective registryTJCExcellent interMRSSSJCSclerosisContent validityScoresCountRheumatologistsPatientsRegistryCohortAbnormalities
2016
Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial
Tashkin DP, Roth MD, Clements PJ, Furst DE, Khanna D, Kleerup EC, Goldin J, Arriola E, Volkmann ER, Kafaja S, Silver R, Steen V, Strange C, Wise R, Wigley F, Mayes M, Riley DJ, Hussain S, Assassi S, Hsu VM, Patel B, Phillips K, Martinez F, Golden J, Connolly MK, Varga J, Dematte J, Hinchcliff ME, Fischer A, Swigris J, Meehan R, Theodore A, Simms R, Volkov S, Schraufnagel DE, Scholand MB, Frech T, Molitor JA, Highland K, Read CA, Fritzler MJ, Kim GHJ, Tseng CH, Elashoff RM, Investigators S. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. The Lancet Respiratory Medicine 2016, 4: 708-719. PMID: 27469583, PMCID: PMC5014629, DOI: 10.1016/s2213-2600(16)30152-7.Peer-Reviewed Original ResearchConceptsInterstitial lung diseaseMycophenolate mofetilParallel-group trialLung diseaseOral cyclophosphamidePrimary endpointCyclophosphamide groupGroup trialsPrimary analysisProgressive interstitial lung diseaseMycophenolate mofetil groupUS medical centersTreatment of sclerodermaProgression of sclerodermaPotential clinical effectivenessModified intentionProgressive sclerodermaStudy drugGood tolerabilityHRCT studiesLung functionPulmonary functionTreat analysisTreatment failureVital capacityTenascin-C drives persistence of organ fibrosis
Bhattacharyya S, Wang W, Morales-Nebreda L, Feng G, Wu M, Zhou X, Lafyatis R, Lee J, Hinchcliff M, Feghali-Bostwick C, Lakota K, Budinger GR, Raparia K, Tamaki Z, Varga J. Tenascin-C drives persistence of organ fibrosis. Nature Communications 2016, 7: 11703. PMID: 27256716, PMCID: PMC4895803, DOI: 10.1038/ncomms11703.Peer-Reviewed Original ResearchConceptsSystemic sclerosisToll-like receptorsOrgan fibrosisFibrosis resolutionPathogenesis of SScTreatment of SScLevels of tenascinEndogenous danger signalsSSc skin biopsy samplesSkin biopsy samplesMechanism of actionLung fibrosisPathogenic roleTLR activatorsMouse modelBiopsy samplesFibroblast activationDanger signalsMyofibroblast transformationFibrosisSSc fibroblastsCollagen gene expressionSkin fibroblastsAmplification loopTenascin