2023
Three Distinct Transcriptional Profiles of Monocytes Associate with Disease Activity in Scleroderma Patients
Makinde H, Dunn JLM, Gadhvi G, Carns M, Aren K, Chung AH, Muhammad LN, Song J, Cuda CM, Dominguez S, Pandolfino JE, D'Amico J, Budinger GS, Assassi S, Frech TM, Khanna D, Shaeffer A, Perlman H, Hinchcliff M, Winter DR. Three Distinct Transcriptional Profiles of Monocytes Associate with Disease Activity in Scleroderma Patients. Arthritis & Rheumatology 2023, 75: 595-608. PMID: 36281773, PMCID: PMC10165944, DOI: 10.1002/art.42380.Peer-Reviewed Original ResearchMeSH KeywordsBiomarkersHumansMacrophagesMonocytesScleroderma, DiffuseScleroderma, LocalizedScleroderma, SystemicSkinConceptsDiffuse cutaneous systemic sclerosisNonclassical monocytesClassical monocytesSSc patientsTranscriptional signatureSystemic sclerosisSkin diseasesCharacteristic transcriptional signaturesRNA-seq dataSSc skin diseaseWorse lung functionCutaneous systemic sclerosisEarly systemic sclerosisActivity outcome measuresDistinct transcriptional profilesTranscriptional profilingRNA sequencingTranscriptional profilesProspective registryDisease activityLung functionComplex clinical phenotypeGene expressionPeripheral bloodMinority cell population
2021
Clinical characteristics, visceral involvement, and mortality in at-risk or early diffuse systemic sclerosis: a longitudinal analysis of an observational prospective multicenter US cohort
Jaafar S, Lescoat A, Huang S, Gordon J, Hinchcliff M, Shah AA, Assassi S, Domsic R, Bernstein EJ, Steen V, Elliott S, Hant F, Castelino FV, Shanmugam VK, Correia C, Varga J, Nagaraja V, Roofeh D, Frech T, Khanna D. Clinical 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 & Therapy 2021, 23: 170. PMID: 34127049, PMCID: PMC8201684, DOI: 10.1186/s13075-021-02548-1.Peer-Reviewed Original ResearchMeSH KeywordsCohort StudiesHumansLung Diseases, InterstitialMycophenolic AcidProspective StudiesScleroderma, DiffuseScleroderma, SystemicUnited StatesConceptsDiffuse cutaneous systemic sclerosisInterstitial lung diseaseImmunosuppressive therapyProgression of skinCardiac involvementSystemic sclerosisUS cohortAnti-RNA polymerase III antibodiesProgressive interstitial lung diseaseEarly diffuse systemic sclerosisMean HAQ-DIMulticenter prospective cohortMulticenter US cohortVital capacity declineMedian disease durationCurrent immunosuppressive therapiesCutaneous systemic sclerosisInternal organ involvementPolymerase III antibodiesDiffuse systemic sclerosisLimited cutaneous SScHigh case fatalityLongitudinal analysisBaseline mRSHAQ-DI
2020
Performance 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 Research
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 course
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
The relationship between skin symptoms and the scleroderma modification of the health assessment questionnaire, the modified Rodnan skin score, and skin pathology in patients with systemic sclerosis
Ziemek J, Man A, Hinchcliff M, Varga J, Simms RW, Lafyatis R. The relationship between skin symptoms and the scleroderma modification of the health assessment questionnaire, the modified Rodnan skin score, and skin pathology in patients with systemic sclerosis. Rheumatology 2016, 55: 911-917. PMID: 26880832, PMCID: PMC5854039, DOI: 10.1093/rheumatology/kew003.Peer-Reviewed Original ResearchConceptsRodnan skin scoreHealth Assessment QuestionnaireSkin symptomsSkindex-29Skin scoreAssessment QuestionnaireSkin diseasesSkin pathologySSc skin diseaseSymptom assessment questionnairePatient's skin symptomsSSc patientsSystemic sclerosisAtopic dermatitisPathological featuresSymptom assessmentPhysical examMyofibroblast infiltrationPathological changesPathological measuresPatientsSymptom instrumentsSymptomsDiseaseSHAQ
2015
Cardiac metabolomics and autopsy in a patient with early diffuse systemic sclerosis presenting with dyspnea: a case report
Frech TM, Revelo MP, Ryan JJ, Shah AA, Gordon J, Domsic R, Hant F, Assassi S, Shanmugam VK, Hinchcliff M, Steen V, Khanna D, Bernstein EJ, Cox J, Luem N, Drakos S. Cardiac metabolomics and autopsy in a patient with early diffuse systemic sclerosis presenting with dyspnea: a case report. Journal Of Medical Case Reports 2015, 9: 136. PMID: 26055398, PMCID: PMC4469401, DOI: 10.1186/s13256-015-0587-7.Peer-Reviewed Original ResearchMeSH KeywordsAutopsyDyspneaFatal OutcomeFemaleHumansHypertension, PulmonaryMetabolomicsMiddle AgedMyocardiumScleroderma, DiffuseConceptsDiffuse systemic sclerosisSystemic sclerosisCardiac deathCase reportTissue metabolomicsEarly diffuse systemic sclerosisCardiac magnetic resonance imagingRight heart catheterizationPulmonary function testsCaucasian female patientSystemic sclerosis pathogenesisMagnetic resonance imagingRenal crisisHeart catheterizationCase presentationADigital ulcerationFemale patientsFunction testsSkin thickeningRare diseaseDisease pathogenesisHigh mortalityResonance imagingDyspneaSclerosis
2014
Pulmonary hypertension and interstitial lung disease within PHAROS: impact of extent of fibrosis and pulmonary physiology on cardiac haemodynamic parameters.
Fischer A, Swigris J, Bolster M, Chung L, Csuka M, Domsic R, Frech T, Hinchcliff M, Hsu V, Hummers L, Gomberg-Maitland M, Mathai S, Simms R, Steen V. Pulmonary hypertension and interstitial lung disease within PHAROS: impact of extent of fibrosis and pulmonary physiology on cardiac haemodynamic parameters. Clinical And Experimental Rheumatology 2014, 32: s-109-14. PMID: 25372796.Peer-Reviewed Original ResearchConceptsSeverity of ILDHaemodynamic parametersNew York Heart Association functional class IIFunctional class IILeft heart diseasePulmonary vascular diseasePulmonary function testingInterstitial lung diseaseImpact of extentMajority of subjectsHRCT evidenceILD extentILD severityPH-ILDSSc-ILDPulmonary hypertensionBaseline characteristicsHaemodynamic profileCutaneous SScHRCT scansFunction testingLung diseaseVascular diseaseHeart diseasePulmonary physiology
2013
Treatment of early diffuse systemic sclerosis skin disease.
Frech TM, Shanmugam VK, Shah AA, Assassi S, Gordon JK, Hant FN, Hinchcliff ME, Steen V, Khanna D, Kayser C, Domsic RT. Treatment of early diffuse systemic sclerosis skin disease. Clinical And Experimental Rheumatology 2013, 31: 166-71. PMID: 23910619, PMCID: PMC3889109.Peer-Reviewed Original ResearchConceptsSSc skin diseaseSystemic sclerosisSkin diseasesEarly diffuse cutaneous systemic sclerosisDiffuse cutaneous systemic sclerosisEarly diffuse SScEarly systemic sclerosisCutaneous systemic sclerosisDiffuse systemic sclerosisIncident cohort studyIntravenous immunoglobulinProspective registryCohort studyInter-institutional variabilityClinical courseHigh morbidityDiffuse SScEffective treatmentNovel biomarkersDisease pathogenesisSclerosisDiseaseTreatmentRigorous studiesMycophenolate