2023
Prognostic significance of pericardial effusion in systemic sclerosis-associated pulmonary hypertension: analysis from the PHAROS Registry
Luo Y, Gordon J, Xu J, Kolstad K, Chung L, Steen V, Bernstein E, Csuka M, Derk C, Domsic R, Fischer A, Frech T, Goldberg A, Hant F, Hinchcliff M, Horn E, Hummers L, Hsu V, Kafaja S, Khanna D, Molitor J, Saketkoo L, Shapiro L, Silver R, Simms R. Prognostic significance of pericardial effusion in systemic sclerosis-associated pulmonary hypertension: analysis from the PHAROS Registry. Rheumatology 2023, 63: 1251-1258. PMID: 37478347, PMCID: PMC11065440, DOI: 10.1093/rheumatology/kead368.Peer-Reviewed Original ResearchPersistent pericardial effusionPericardial effusionPulmonary hypertensionSSc-PHSystemic sclerosisPrognostic significanceEffusion statusSystemic sclerosis-associated pulmonary hypertensionMultivariable Cox proportional hazards regressionCox proportional hazards regressionKaplan-Meier survival analysisProspective multicentre registryPulmonary Hypertension AssessmentRight heart catheterizationPredictors of survivalMultivariable-adjusted analysesProportional hazards regressionLog-rank testRecognition of OutcomesMulticentre registryPrognosticate survivalHeart catheterizationIndependent predictorsPrognostic impactSerial echocardiograms
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
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 ResearchConceptsPulmonary 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 ResearchConceptsDiffuse 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 scoreSurvival 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
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