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
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 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 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 patients
2008
Systemic sclerosis/scleroderma: a treatable multisystem disease.
Hinchcliff M, Varga J. Systemic sclerosis/scleroderma: a treatable multisystem disease. American Family Physician 2008, 78: 961-8. PMID: 18953973.Peer-Reviewed Original ResearchConceptsInternal organ involvementSystemic sclerosisOrgan involvementRaynaud's phenomenonEndothelin-1 receptor blockerChronic connective tissue diseaseEnd-organ dysfunctionEnzyme inhibitor therapyHigh-dose corticosteroidsScleroderma renal crisisPulmonary arterial hypertensionPulmonary function testingConnective tissue diseaseDisease-modifying therapiesDistinct clinical subsetInternal organ dysfunctionOrgan-specific manifestationsPhosphodiesterase-5 inhibitorsMiddle-age womenOptimal patient careWidespread microvascular damageRenal crisisArterial hypertensionDigital ulcersLifestyle modification