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 physiology