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
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 peptide
2016
Utility of B-type natriuretic peptides in the assessment of patients with systemic sclerosis-associated pulmonary hypertension in the PHAROS registry.
Chung L, Fairchild RM, Furst DE, Li S, Alkassab F, Bolster MB, Csuka ME, Derk CT, Domsic RT, Fischer A, Frech TM, Gomberg-Maitland M, Gordon JK, Hinchcliff M, Hsu V, Hummers LK, Khanna D, Medsger TAJ, Molitor JA, Preston IR, Schiopu E, Shapiro L, Hant F, Silver R, Simms R, Varga J, Steen VD, Zamanian RT. Utility of B-type natriuretic peptides in the assessment of patients with systemic sclerosis-associated pulmonary hypertension in the PHAROS registry. Clinical And Experimental Rheumatology 2016, 35 Suppl 106: 106-113. PMID: 27908301.Peer-Reviewed Original ResearchConceptsB-type natriuretic peptideNT-proBNP levelsPg/mLSSc-PHNT-proBNPPulmonary hypertensionSSc patientsSystemic sclerosisNatriuretic peptideBaseline B-type natriuretic peptideMedian B-type natriuretic peptideSystemic sclerosis-associated pulmonary hypertensionMulticenter prospective cohortNT-proBNP groupNatriuretic peptide levelsRight ventricular dysfunctionPredictors of mortalityPulmonary arterial hypertensionPredictors of progressionAssessment of patientsBaseline creatinineSSc-PAHArterial hypertensionPAH patientsVentricular dysfunctionCalcinosis is associated with digital ulcers and osteoporosis in patients with systemic sclerosis: A Scleroderma Clinical Trials Consortium study
Valenzuela A, Baron M, Group C, Herrick AL, Proudman S, Stevens W, Group A, Rodriguez-Reyna TS, Vacca A, Medsger TA, Hinchcliff M, Hsu V, Wu JY, Fiorentino D, Chung L. Calcinosis is associated with digital ulcers and osteoporosis in patients with systemic sclerosis: A Scleroderma Clinical Trials Consortium study. Seminars In Arthritis And Rheumatism 2016, 46: 344-349. PMID: 27371996, PMCID: PMC5500288, DOI: 10.1016/j.semarthrit.2016.05.008.Peer-Reviewed Original ResearchMeSH KeywordsAcro-OsteolysisAdultAgedAntibodies, AntinuclearAutoantibodiesCalcinosisCohort StudiesDNA Topoisomerases, Type IFemaleFingersHeart DiseasesHumansHypertension, PulmonaryLogistic ModelsMaleMiddle AgedMultivariate AnalysisNuclear ProteinsOdds RatioOsteoporosisRetrospective StudiesRibonucleoprotein, U1 Small NuclearRNA Polymerase IIIScleroderma, SystemicSkin UlcerTelangiectasisConceptsScleroderma Clinical Trials ConsortiumDigital ulcersSystemic sclerosisOdds ratioMultivariate analysisAnti-PM/SclRetrospective cohort studyQuarter of patientsLonger disease durationClinical Trials ConsortiumStrong associationMulticenter collaborative effortsGastrointestinal involvementAnticardiolipin antibodiesPulmonary hypertensionCohort studyDisease durationClinical factorsAcro-osteolysisClinical featuresUnivariate analysisCardiac diseasePatientsCalcinosisUlcers
2015
Survival in systemic sclerosis–pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry
Hinchcliff M, Khanna S, Hsu VM, Lee J, Almagor O, Chang RW, Steen V, Chung L, Investigators T. Survival in systemic sclerosis–pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry. Seminars In Arthritis And Rheumatism 2015, 45: 309-314. PMID: 26210782, PMCID: PMC4656087, DOI: 10.1016/j.semarthrit.2015.06.011.Peer-Reviewed Original ResearchMeSH KeywordsAdultAgedAutoantibodiesFemaleHumansHypertension, PulmonaryMaleMiddle AgedPrognosisRegistriesScleroderma, SystemicConceptsPulmonary arterial hypertensionPulmonary Hypertension AssessmentSerum autoantibodiesRecognition of OutcomesScleroderma RegistryArterial hypertensionScl-70Hypertension assessmentAutoantibody groupsSystemic sclerosis-pulmonary arterial hypertensionSocial Security Death IndexKaplan-Meier survival estimatesSurvival estimatesIncident systemic sclerosisSSc disease durationSSc-PAH patientsRight heart catheterizationRisk of deathProportional hazards modelClinical laboratoriesElectronic medical recordsMedian followSSc-PAHAutoantibody statusHeart catheterizationCardiac 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
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 scorePulmonary 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 physiologyDevelopment of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study
Hsu VM, Chung L, Hummers LK, Wigley F, Simms R, Bolster M, Silver R, 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, Alkassab F, Steen VD. Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study. Seminars In Arthritis And Rheumatism 2014, 44: 55-62. PMID: 24709277, DOI: 10.1016/j.semarthrit.2014.03.002.Peer-Reviewed Original ResearchMeSH KeywordsAdultAgedFemaleHumansHypertension, PulmonaryLongitudinal StudiesMaleMiddle AgedProspective StudiesRaynaud DiseaseRegistriesRiskScleroderma, SystemicConceptsSystolic pulmonary arterial pressureRight heart catheterizationExercise-induced hypoxiaPulmonary hypertensionPulmonary arterial hypertensionPulmonary function testingSystemic sclerosisCohort studyDisease durationHigher systolic pulmonary arterial pressureProspective longitudinal cohort studyFVC/DLcoNew pulmonary hypertensionPulmonary Hypertension AssessmentDiffusion lung capacityPulmonary arterial pressureOngoing prospective studyKaplan-Meier survivalHigh-risk populationKaplan-Meier estimatesLongitudinal cohort studyHigh-risk factorsRecognition of OutcomesPFT criteriaPH patientsSurvival and Predictors of Mortality in Systemic Sclerosis‐Associated Pulmonary Arterial Hypertension: Outcomes From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry
Chung L, Domsic RT, Lingala B, Alkassab F, Bolster M, Csuka ME, Derk C, Fischer A, Frech T, Furst DE, Gomberg‐Maitland M, Hinchcliff M, Hsu V, Hummers LK, Khanna D, Medsger TA, Molitor JA, Preston IR, Schiopu E, Shapiro L, Silver R, Simms R, Varga J, Gordon JK, Steen VD. Survival and Predictors of Mortality in Systemic Sclerosis‐Associated Pulmonary Arterial Hypertension: Outcomes From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry. Arthritis Care & Research 2014, 66: 489-495. PMID: 23983198, DOI: 10.1002/acr.22121.Peer-Reviewed Original ResearchConceptsPulmonary arterial hypertensionPulmonary Hypertension AssessmentCumulative survival rateRecognition of OutcomesSurvival rateScleroderma RegistryArterial hypertensionSSc patientsHypertension assessmentRoutine screeningWorld Health Organization group I PAHSystemic sclerosis associated pulmonary arterial hypertensionGroup I pulmonary arterial hypertensionSSc-associated pulmonary arterial hypertensionIncident pulmonary arterial hypertensionSSc centersRight-sided heart catheterizationDefinite pulmonary hypertensionIncident systemic sclerosisPredictors of mortalityMonths of enrollmentHeart catheterizationPAH diagnosisProspective registryPulmonary hypertension
2012
Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry
Bae S, Saggar R, Bolster MB, Chung L, Csuka ME, Derk C, Domsic R, Fischer A, Frech T, Goldberg A, Hinchcliff M, Hsu V, Hummers L, Schiopu E, Mayes MD, McLaughlin V, Molitor J, Naz N, Furst DE, Maranian P, Steen V, Khanna D. Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry. Annals Of The Rheumatic Diseases 2012, 71: 1335. PMID: 22307943, PMCID: PMC3398226, DOI: 10.1136/annrheumdis-2011-200546.Peer-Reviewed Original ResearchConceptsRight heart catheterisationPulmonary hypertensionSystemic sclerosisHigh-resolution CTNormal haemodynamicsBaseline characteristicsBorderline mean pulmonary arterial pressureHigh pulmonary vascular resistanceMean pulmonary arterial pressureRight ventricular systolic pressureAbnormal lung physiologyBaseline clinical characteristicsMean pulmonary pressureProspective longitudinal cohortPulmonary vascular resistancePulmonary arterial pressurePulmonary function testsRestrictive lung diseaseVentricular systolic pressureLong-term outcomesElevated mPAPHeart catheterisationVascular resistanceBaseline demographicsClinical characteristics
2011
Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): Baseline Characteristics and Description of Study Population
Hinchcliff M, Fischer A, Schiopu E, Steen VD. Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): Baseline Characteristics and Description of Study Population. The Journal Of Rheumatology 2011, 38: 2172-2179. PMID: 21844142, PMCID: PMC3230328, DOI: 10.3899/jrheum.101243.Peer-Reviewed Original ResearchConceptsPulmonary arterial hypertensionIncident pulmonary hypertensionPulmonary hypertensionSystemic sclerosisHigh riskBaseline characteristicsDevelopment of PAHEstimated right ventricular systolic pressureIncident pulmonary arterial hypertensionLeft-side heart diseaseRight ventricular systolic pressureCarbon monoxide diffusing capacityPAH-specific therapyPulmonary Hypertension AssessmentRight heart catheterizationPulmonary venous hypertensionVentricular systolic pressureHigh-risk subjectsInterstitial lung diseaseRecognition of OutcomesSSc-PAHArterial hypertensionHeart catheterizationVenous hypertensionSSc patients