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
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 catheterization
2014
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
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 ResearchConceptsSystolic 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
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