2021
Clinical characteristics, visceral involvement, and mortality in at-risk or early diffuse systemic sclerosis: a longitudinal analysis of an observational prospective multicenter US cohort
Jaafar S, Lescoat A, Huang S, Gordon J, Hinchcliff M, Shah AA, Assassi S, Domsic R, Bernstein EJ, Steen V, Elliott S, Hant F, Castelino FV, Shanmugam VK, Correia C, Varga J, Nagaraja V, Roofeh D, Frech T, Khanna D. Clinical characteristics, visceral involvement, and mortality in at-risk or early diffuse systemic sclerosis: a longitudinal analysis of an observational prospective multicenter US cohort. Arthritis Research & Therapy 2021, 23: 170. PMID: 34127049, PMCID: PMC8201684, DOI: 10.1186/s13075-021-02548-1.Peer-Reviewed Original ResearchConceptsDiffuse cutaneous systemic sclerosisInterstitial lung diseaseImmunosuppressive therapyProgression of skinCardiac involvementSystemic sclerosisUS cohortAnti-RNA polymerase III antibodiesProgressive interstitial lung diseaseEarly diffuse systemic sclerosisMean HAQ-DIMulticenter prospective cohortMulticenter US cohortVital capacity declineMedian disease durationCurrent immunosuppressive therapiesCutaneous systemic sclerosisInternal organ involvementPolymerase III antibodiesDiffuse systemic sclerosisLimited cutaneous SScHigh case fatalityLongitudinal analysisBaseline mRSHAQ-DI
2019
Global skin gene expression analysis of early diffuse cutaneous systemic sclerosis shows a prominent innate and adaptive inflammatory profile
Skaug B, Khanna D, Swindell WR, Hinchcliff ME, Frech TM, Steen VD, Hant FN, Gordon JK, Shah AA, Zhu L, Zheng WJ, Browning JL, Barron AMS, Wu M, Visvanathan S, Baum P, Franks JM, Whitfield ML, Shanmugam VK, Domsic RT, Castelino FV, Bernstein EJ, Wareing N, Lyons MA, Ying J, Charles J, Mayes MD, Assassi S. Global skin gene expression analysis of early diffuse cutaneous systemic sclerosis shows a prominent innate and adaptive inflammatory profile. Annals Of The Rheumatic Diseases 2019, 79: annrheumdis-2019-215894. PMID: 31767698, PMCID: PMC7386329, DOI: 10.1136/annrheumdis-2019-215894.Peer-Reviewed Original ResearchConceptsImmune cell signaturesEarly diffuse systemic sclerosisDiffuse systemic sclerosisLonger disease durationSystemic sclerosisDisease durationCell signatureSSc patientsT cellsEarly diffuse cutaneous systemic sclerosisScleroderma Outcome Study cohortDiffuse cutaneous systemic sclerosisShorter disease durationCutaneous systemic sclerosisRodnan skin scoreCD8 T cellsB cell signaturesCD4 T cellsSystemic sclerosis cohortImmune cell markersClinical trial designDiffuse SSc patientsBiopsy RNAProspective registryClinical course
2018
SAT0486 Myeloablation followed by autologous stem cell rescue leads to normalisation of serum il-6 levels in patients with systemic sclerosis
Bellocchi C, Sullivan K, Goldmuntz E, Ying J, Keyes-Elstein L, Varga J, Hinchcliff M, McSweeney P, Furst D, Nash R, Crofford L, Welch B, Pinckney A, Mayes M, Assassi S. SAT0486 Myeloablation followed by autologous stem cell rescue leads to normalisation of serum il-6 levels in patients with systemic sclerosis. Annals Of The Rheumatic Diseases 2018, 77: 1100. DOI: 10.1136/annrheumdis-2018-eular.3731.Peer-Reviewed Original ResearchSerum IL-6 levelsHematopoietic stem cell transplantationIL-6 levelsStem cell transplantationSystemic sclerosisIL-6IL-12IL-1βSCOT trialDisease durationSSc pathogenesisAutologous hematopoietic stem cell transplantationPro-inflammatory Th1 cytokinesAutologous stem cell rescueAutologous stem cell transplantationSerum IFN-γ levelsBaseline clinical featuresMean disease durationStem cell rescueRodnan skin scoreSerum IL-6Diffuse systemic sclerosisMajority of patientsAvailable serum samplesIFN-γ levels
2015
Cardiac 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
2013
Treatment of early diffuse systemic sclerosis skin disease.
Frech TM, Shanmugam VK, Shah AA, Assassi S, Gordon JK, Hant FN, Hinchcliff ME, Steen V, Khanna D, Kayser C, Domsic RT. Treatment of early diffuse systemic sclerosis skin disease. Clinical And Experimental Rheumatology 2013, 31: 166-71. PMID: 23910619, PMCID: PMC3889109.Peer-Reviewed Original ResearchConceptsSSc skin diseaseSystemic sclerosisSkin diseasesEarly diffuse cutaneous systemic sclerosisDiffuse cutaneous systemic sclerosisEarly diffuse SScEarly systemic sclerosisCutaneous systemic sclerosisDiffuse systemic sclerosisIncident cohort studyIntravenous immunoglobulinProspective registryCohort studyInter-institutional variabilityClinical courseHigh morbidityDiffuse SScEffective treatmentNovel biomarkersDisease pathogenesisSclerosisDiseaseTreatmentRigorous studiesMycophenolate