2023
Clinical Phenotypes of Patients With Systemic Sclerosis With Distinct Molecular Signatures in Skin
Yang M, Goh V, Lee J, Espinoza M, Yuan Y, Carns M, Aren K, Chung L, Khanna D, McMahan ZH, Agrawal R, Nelson L, Shah SJ, Whitfield ML, Hinchcliff M. Clinical Phenotypes of Patients With Systemic Sclerosis With Distinct Molecular Signatures in Skin. Arthritis Care & Research 2023, 75: 1469-1480. PMID: 35997480, PMCID: PMC9947190, DOI: 10.1002/acr.24998.Peer-Reviewed Original ResearchConceptsRadiographic interstitial lung diseaseInterstitial lung diseaseHigher mRSSSSc patientsSerum autoantibodiesClinical phenotypeHealthy participantsScl-70 antibodyPresent multicenter studySimilar disease durationSystemic sclerosis patientsDistinct clinical phenotypesSkin gene expressionValuable clinical informationDcSSc patientsDisease durationIdentifies patientsSystemic sclerosisSclerosis patientsMulticenter studyLung diseaseSkin fibrosisSimilar prevalenceClinical dataMultivariable modelingHyperspectral imaging in systemic sclerosis-associated Raynaud phenomenon
Teaw S, Gupta A, Williams A, Wilson F, Sumpio B, Sumpio B, Hinchcliff M. Hyperspectral imaging in systemic sclerosis-associated Raynaud phenomenon. Arthritis Research & Therapy 2023, 25: 10. PMID: 36670487, PMCID: PMC9854186, DOI: 10.1186/s13075-023-02990-3.Peer-Reviewed Original ResearchConceptsRaynaud's condition scoreCochin Hand Function ScaleSSc patientsClinic settingPatient-reported outcome instrumentsResultsNinety-two percentSystemic sclerosis patientsMann-Whitney U testHealthy control participantsCHF scoreSSc durationSSc-RPDigital ulcersCutaneous SScProvocation challengeRaynaud's phenomenonSclerosis patientsClinical trialsOutcome instrumentsOxygen saturationBurden assessmentPatientsCold provocationHemoglobin concentrationDisease severity
2020
Regulator combinations identify systemic sclerosis patients with more severe disease
Wang Y, Franks JM, Yang M, Toledo DM, Wood TA, Hinchcliff M, Whitfield ML. Regulator combinations identify systemic sclerosis patients with more severe disease. JCI Insight 2020, 5: e137567. PMID: 32721949, PMCID: PMC7526449, DOI: 10.1172/jci.insight.137567.Peer-Reviewed Original ResearchConceptsInterstitial lung diseaseSystemic sclerosisActivity scoreSevere diseaseSevere interstitial lung diseaseSubsets of SScVital capacity declineRodnan skin scoreSubgroup of patientsSystemic sclerosis patientsHeterogeneous autoimmune disorderInternal organ dysfunctionGene expression subsetsSkin gene expressionActivation of regulatorsIntrinsic subsetOrgan dysfunctionSclerosis patientsSkin scoreAutoantibody productionClinical outcomesAutoimmune disordersClinical variablesLung diseaseSkin fibrosis
2018
Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients At Risk for Pulmonary Hypertension, in the PHAROS Registry
Hsu VM, Chung L, Hummers LK, Shah A, Simms R, Bolster M, Hant FN, Silver RM, Fischer A, Hinchcliff ME, Varga J, Goldberg AZ, Derk CT, Schiopu E, Khanna D, Shapiro LS, Domsic RT, Medsger T, Mayes MD, Furst D, Csuka ME, Molitor JA, Saketkoo LA, Salazar CR, Steen VD. Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients At Risk for Pulmonary Hypertension, in the PHAROS Registry. The Journal Of Rheumatology 2018, 46: 176-183. PMID: 30275260, DOI: 10.3899/jrheum.180018.Peer-Reviewed Original ResearchConceptsExercise oxygen desaturationPulmonary hypertensionCardiopulmonary hospitalizationsPericardial effusionSystemic sclerosisOxygen desaturationMale sexRisk factorsSSc-pulmonary arterial hypertensionSystolic pulmonary arterial pressurePredictors of hospitalizationRight heart catheterizationPredictors of mortalityProspective cohort studyPulmonary arterial pressurePulmonary function testingSystemic sclerosis patientsBaseline clinical measuresProportional hazards modelPH patientsArterial hypertensionDyspnea scoreHeart catheterizationCohort studyDisease duration
2017
Abnormal esophageal acid exposure on high‐dose proton pump inhibitor therapy is common in systemic sclerosis patients
Stern EK, Carlson DA, Falmagne S, Hoffmann AD, Carns M, Pandolfino JE, Hinchcliff M, Brenner DM. Abnormal esophageal acid exposure on high‐dose proton pump inhibitor therapy is common in systemic sclerosis patients. Neurogastroenterology & Motility 2017, 30 PMID: 29110377, PMCID: PMC5771836, DOI: 10.1111/nmo.13247.Peer-Reviewed Original ResearchConceptsProton pump inhibitorsAcid exposure timeEsophageal acid exposureSystemic sclerosis patientsHigh-dose proton pump inhibitorsEsophageal pH-impedance testingGastro-esophageal reflux diseasePH-impedance testingSSc patientsSystemic sclerosisSclerosis patientsAcid exposurePPI therapyHigh-dose proton pump inhibitor therapyAbnormal esophageal acid exposureCase-control retrospective analysisHigh-dose PPI therapyHigh-resolution manometry findingsHigher acid exposure timeTotal acid exposure timeProton pump inhibitor therapyTwice-daily proton pump inhibitorsHiatal hernia sizeNon-SSc patientsRheumatology SSc criteria
2015
Experimentally-Derived Fibroblast Gene Signatures Identify Molecular Pathways Associated with Distinct Subsets of Systemic Sclerosis Patients in Three Independent Cohorts
Johnson ME, Mahoney JM, Taroni J, Sargent JL, Marmarelis E, Wu MR, Varga J, Hinchcliff ME, Whitfield ML. Experimentally-Derived Fibroblast Gene Signatures Identify Molecular Pathways Associated with Distinct Subsets of Systemic Sclerosis Patients in Three Independent Cohorts. PLOS ONE 2015, 10: e0114017. PMID: 25607805, PMCID: PMC4301872, DOI: 10.1371/journal.pone.0114017.Peer-Reviewed Original ResearchConceptsNormal-like subsetsGenome-wide expression profilingSet of genesDifferential gene expressionDistinct signaling pathwaysInflammatory subsetSystemic sclerosisGene signatureIndependent cohortGene expression signaturesLipid signalingInnate immune pathwaysActive TGFβ signalingExpression profilingSystemic sclerosis patientsMolecular relationshipsGene expressionNon-lesional skinTGFβ signalingSignaling pathwaysSubsets of diseaseMolecular pathwaysMicroarray datasetsExpression signaturesEarly disease pathology
2014
Antinuclear antibody-negative systemic sclerosis
Salazar GA, Assassi S, Wigley F, Hummers L, Varga J, Hinchcliff M, Khanna D, Schiopu E, Phillips K, Furst DE, Steen V, Baron M, Hudson M, Taillefer SS, Pope J, Jones N, Docherty P, Khalidi NA, Robinson D, Simms RW, Silver RM, Frech TM, Fessler BJ, Molitor JA, Fritzler MJ, Segal BM, Al-Kassab F, Perry M, Yang J, Zamanian S, Reveille JD, Arnett FC, Pedroza C, Mayes MD. Antinuclear antibody-negative systemic sclerosis. Seminars In Arthritis And Rheumatism 2014, 44: 680-686. PMID: 25578738, PMCID: PMC4447614, DOI: 10.1016/j.semarthrit.2014.11.006.Peer-Reviewed Original ResearchConceptsANA-negative groupANA-negative patientsPulmonary arterial hypertensionAntinuclear antibodiesSSc patientsDiffuse cutaneous involvementLower gastrointestinal involvementNegative antinuclear antibodyRight heart catheterizationScleroderma Family RegistrySSc-related antibodiesRodnan skin scoreSystemic sclerosis patientsANA-positive patientsRenal crisisVasculopathic manifestationsCause mortalityCutaneous involvementGastrointestinal involvementArterial hypertensionChart reviewClinical characteristicsHeart catheterizationMale patientsSkin score