2024
Systemic sclerosis: beyond skin and lung involvement
Hinchcliff M. Systemic sclerosis: beyond skin and lung involvement. Current Opinion In Rheumatology 2024, 36: 371-373. PMID: 39584403, DOI: 10.1097/bor.0000000000001053.Peer-Reviewed Original ResearchANTAGONISM OF CGAS ABROGATES INFLAMMATORY FIBROTIC RESPONSES IN TRANSLATIONAL MODELS OF SCLERODERMA-ASSOCIATED INTERSTITIAL LUNG DISEASE
YU S, LEE C, HU B, SUN Y, SHAO S, SUN H, GHINCEA A, WOO S, MCGOVERN J, SABER T, GUNES B, KUJAWSKI S, PEREZ S, ODELL W, HINCHCLIFF M, VARGA J, SAULER M, GOMEZ J, RYU C, HERZOG E. ANTAGONISM OF CGAS ABROGATES INFLAMMATORY FIBROTIC RESPONSES IN TRANSLATIONAL MODELS OF SCLERODERMA-ASSOCIATED INTERSTITIAL LUNG DISEASE. CHEST Journal 2024, 166: a3380-a3381. DOI: 10.1016/j.chest.2024.06.2018.Peer-Reviewed Original ResearchGastrointestinal disease in systemic sclerosis: the neglected organ system?
McMahan Z, Pandolfino J, Perlman H, Del Galdo F, Hinchcliff M. Gastrointestinal disease in systemic sclerosis: the neglected organ system? Current Opinion In Rheumatology 2024 PMID: 39193877, DOI: 10.1097/bor.0000000000001052.Peer-Reviewed Original ResearchSystemic sclerosisClinical trial endpointsInflammatory bowel diseaseTrial endpointsAssessment of disease activityRisk stratification methodsGastrointestinal (GIDisease activityRisk stratificationEffective therapyImmune responseBowel diseaseDisease pathogenesisTherapeutic interventionsGastrointestinal diseasesOrgan systemsDiseaseMultidisciplinary teamPathogenesisFunctional studiesGut microbiomeEndpointSclerosisPOS0835 VOLUMETRIC QUANTIFICATION OF SYSTEMIC SCLEROSIS-ASSOCIATED CALCINOSIS CUTIS
Hinchcliff M, Wu M, Odell I, Papademetris X, Feher A, Zhao Y, Lee H, Lempel M, Wang A, Galiana G, Van Horn C, Gunes B, Kujawski S. POS0835 VOLUMETRIC QUANTIFICATION OF SYSTEMIC SCLEROSIS-ASSOCIATED CALCINOSIS CUTIS. 2024, 1041-1042. DOI: 10.1136/annrheumdis-2024-eular.2060.Peer-Reviewed Original ResearchAB1174 VALIDITY OF THE ASSESSMENT OF SYSTEMIC SCLEROSIS – ASSOCIATED RAYNAUD PHENOMENON (ASRAP) QUESTIONNAIRE
Madathanapalli K, Shelton K, Makhijani A, Memon A, Page N, Gunes B, Odell W, Kujawski S, Perez S, Pauling J, Domsic R, Yu L, Wilson F, Hinchcliff M. AB1174 VALIDITY OF THE ASSESSMENT OF SYSTEMIC SCLEROSIS – ASSOCIATED RAYNAUD PHENOMENON (ASRAP) QUESTIONNAIRE. 2024, 1920-1921. DOI: 10.1136/annrheumdis-2024-eular.2331.Peer-Reviewed Original ResearchPOS0824 ARTIFICIAL INTELLIGENCE (DEEP NEURAL NETWORK ANALYSIS) AS A SKIN OUTCOME: RESULTS OF AN OPEN-LABEL BELUMOSUDIL TRIAL IN PATIENTS WITH DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS
Gunes B, Duran Camacho L, Cowper S, Panse G, Page N, Bundschuh E, Williams A, Carns M, Aren K, Pradhan N, Bernstein E, Fantus S, Volkmann E, Bukiri H, Correia C, Wilson F, Mawe S, Mahoney J, Wang R, Hinchcliff M. POS0824 ARTIFICIAL INTELLIGENCE (DEEP NEURAL NETWORK ANALYSIS) AS A SKIN OUTCOME: RESULTS OF AN OPEN-LABEL BELUMOSUDIL TRIAL IN PATIENTS WITH DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS. 2024, 1046.2-1047. DOI: 10.1136/annrheumdis-2024-eular.2892.Peer-Reviewed Original ResearchClustering and Evolving Concepts for Subclassification
Sobanski V, Hinchcliff M. Clustering and Evolving Concepts for Subclassification. 2024, 71-78. DOI: 10.1007/978-3-031-40658-4_6.Peer-Reviewed Original ResearchFactors associated with satisfaction with social roles and activities among people with systemic sclerosis: a Scleroderma Patient-centered Intervention Network (SPIN) cohort cross-sectional study.
Dal Santo T, Rice D, Carrier ME, Virgili-Gervais G, Levis B, Kwakkenbos L, Bartlett SJ, Gietzen A, Gottesman K, Guillot G, Hudson M, Hummers LK, Malcarne V, Mayes M, Mouthon L, Richard M, Sauve M, Wojeck R, Geoffroy MC, Benedetti A, Thombs B, SPIN investigators. Factors associated with satisfaction with social roles and activities among people with systemic sclerosis: a Scleroderma Patient-centered Intervention Network (SPIN) cohort cross-sectional study. RMD Open 2024, 10 PMID: 38428973, DOI: 10.1136/rmdopen-2023-003876.Peer-Reviewed Original ResearchGene expression meta-analysis reveals aging and cellular senescence signatures in scleroderma-associated interstitial lung disease
Yang M, Lee S, Neely J, Hinchcliff M, Wolters P, Sirota M. Gene expression meta-analysis reveals aging and cellular senescence signatures in scleroderma-associated interstitial lung disease. Frontiers In Immunology 2024, 15: 1326922. PMID: 38348044, PMCID: PMC10859856, DOI: 10.3389/fimmu.2024.1326922.Peer-Reviewed Original ResearchConceptsScleroderma-associated interstitial lung diseaseSSc-ILDInterstitial lung diseaseLung tissueGene expression meta-analysisPulmonary fibrosisLung diseaseSenescence signatureDegree of skin involvementIdiopathic pulmonary fibrosisTelomere lengthType II alveolar cellsCellular senescence signaturesCellular senescenceIndependent of ageSkin involvementSSc skinExpression meta-analysisHealthy controlsAssociated with degreeAlveolar cellsLungMeta-analysisAging genesFibrosis
2023
IL-6 trans-signaling in a humanized mouse model of scleroderma
Odell I, Agrawal K, Sefik E, Odell A, Caves E, Kirkiles-Smith N, Horsley V, Hinchcliff M, Pober J, Kluger Y, Flavell R. IL-6 trans-signaling in a humanized mouse model of scleroderma. Proceedings Of The National Academy Of Sciences Of The United States Of America 2023, 120: e2306965120. PMID: 37669366, PMCID: PMC10500188, DOI: 10.1073/pnas.2306965120.Peer-Reviewed Original ResearchConceptsBone marrow-derived immune cellsIL-6Human hematopoietic stem cellsImmune cellsT cellsScleroderma skinSoluble IL-6 receptorCD8 T cellsHumanized mouse modelPathogenesis of sclerodermaMesenchymal cellsFibroblast-derived IL-6IL-6 receptorIL-6 signalingT cell activationHuman IL-6Human T cellsExpression of collagenFibrosis improvementPansclerotic morpheaHuman endothelial cellsHumanized miceReduced markersSkin graftsHuman CD4Prognostic 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 echocardiogramsPOS1258 RESULTS FROM A NOMINAL GROUP TECHNIQUE EXERCISE FOR THE CREATION OF A COMBINED RESPONSE INDEX FOR LIMITED CUTANEOUS SYSTEMIC SCLEROSIS: THE CRISTAL PROJECT
Lescoat A, Chen Y, Murphy S, Carroll L, Vann N, Didio P, Iya S, Phanhdone T, Farrington S, Allanore Y, Cella D, Chung L, Clements P, Del Galdo F, Denton C, Distler O, Hinchcliff M, Hughes M, Hummers L, Pauling J, Pope J, Steen V, Varga J, Buch M, Khanna D. POS1258 RESULTS FROM A NOMINAL GROUP TECHNIQUE EXERCISE FOR THE CREATION OF A COMBINED RESPONSE INDEX FOR LIMITED CUTANEOUS SYSTEMIC SCLEROSIS: THE CRISTAL PROJECT. 2023, 971.2-972. DOI: 10.1136/annrheumdis-2023-eular.4633.Peer-Reviewed Original ResearchClinical Phenotypes of Patients With Systemic Sclerosis With Distinct Molecular Signatures in Skin
Yang M, Goh V, Lee J, Espinoza M, Yuan Y, Carns M, Aren K, Chung L, Khanna D, McMahan ZH, Agrawal R, Nelson L, Shah SJ, Whitfield ML, Hinchcliff M. Clinical Phenotypes of Patients With Systemic Sclerosis With Distinct Molecular Signatures in Skin. Arthritis Care & Research 2023, 75: 1469-1480. PMID: 35997480, PMCID: PMC9947190, DOI: 10.1002/acr.24998.Peer-Reviewed Original ResearchConceptsRadiographic interstitial lung diseaseInterstitial lung diseaseHigher mRSSSSc patientsSerum autoantibodiesClinical phenotypeHealthy participantsScl-70 antibodyPresent multicenter studySimilar disease durationSystemic sclerosis patientsDistinct clinical phenotypesSkin gene expressionValuable clinical informationDcSSc patientsDisease durationIdentifies patientsSystemic sclerosisSclerosis patientsMulticenter studyLung diseaseSkin fibrosisSimilar prevalenceClinical dataMultivariable modelingImpaired left-ventricular global longitudinal strain by feature-tracking cardiac MRI predicts mortality in systemic sclerosis
Feher A, Miller E, Peters D, Mojibian H, Sinusas A, Hinchcliff M, Baldassarre L. Impaired left-ventricular global longitudinal strain by feature-tracking cardiac MRI predicts mortality in systemic sclerosis. Rheumatology International 2023, 43: 849-858. PMID: 36894756, DOI: 10.1007/s00296-023-05294-6.Peer-Reviewed Original ResearchConceptsLate gadolinium enhancementLV-GLSSystemic sclerosisWorse survivalClinical indicationsImpaired left ventricular global longitudinal strainLeft ventricular global longitudinal strainPresence of LGECMR strainLV cardiac indexWorse LV-GLSLV ejection fractionCox regression analysisGlobal longitudinal strainLV cardiac outputGlobal radialCardiac indexOverall survivalRetrospective cohortEjection fractionPrognostic valueAdverse outcomesCardiac outputGadolinium enhancementRV strainA Novel MIP-1-Expressing Macrophage Subtype in BAL Fluid from Healthy Volunteers.
Reyfman PA, Malsin ES, Khuder B, Joshi N, Gadhvi G, Flozak AS, Carns MA, Aren K, Goldberg IA, Kim S, Alexander M, Sporn PHS, Misharin AV, Budinger GRS, Lam AP, Hinchcliff M, Gottardi CJ, Winter DR. A Novel MIP-1-Expressing Macrophage Subtype in BAL Fluid from Healthy Volunteers. American Journal Of Respiratory Cell And Molecular Biology 2023, 68: 176-185. PMID: 36174229, PMCID: PMC9986555, DOI: 10.1165/rcmb.2021-0123oc.Peer-Reviewed Original ResearchConceptsSingle-cell genomic technologiesCell typesSet of genesSingle-cell approachesSingle-cell RNASingle-cell dataSingle-cell studiesAmbient RNAGenomic technologiesMarker genesGene expressionCellular heterogeneityCellular environmentProtein 1RNAMacrophage heterogeneityGenesValuable resourceDistinct subpopulationsMacrophage subtypesHealthy volunteersLung environmentMacrophage subpopulationsMacrophage inflammatory protein-1Inflammatory protein-1Three Distinct Transcriptional Profiles of Monocytes Associate with Disease Activity in Scleroderma Patients
Makinde H, Dunn JLM, Gadhvi G, Carns M, Aren K, Chung AH, Muhammad LN, Song J, Cuda CM, Dominguez S, Pandolfino JE, D'Amico J, Budinger GS, Assassi S, Frech TM, Khanna D, Shaeffer A, Perlman H, Hinchcliff M, Winter DR. Three Distinct Transcriptional Profiles of Monocytes Associate with Disease Activity in Scleroderma Patients. Arthritis & Rheumatology 2023, 75: 595-608. PMID: 36281773, PMCID: PMC10165944, DOI: 10.1002/art.42380.Peer-Reviewed Original ResearchConceptsDiffuse cutaneous systemic sclerosisNonclassical monocytesClassical monocytesSSc patientsTranscriptional signatureSystemic sclerosisSkin diseasesCharacteristic transcriptional signaturesRNA-seq dataSSc skin diseaseWorse lung functionCutaneous systemic sclerosisEarly systemic sclerosisActivity outcome measuresDistinct transcriptional profilesTranscriptional profilingRNA sequencingTranscriptional profilesProspective registryDisease activityLung functionComplex clinical phenotypeGene expressionPeripheral bloodMinority cell populationHyperspectral imaging in systemic sclerosis-associated Raynaud phenomenon
Teaw S, Gupta A, Williams A, Wilson F, Sumpio B, Sumpio B, Hinchcliff M. Hyperspectral imaging in systemic sclerosis-associated Raynaud phenomenon. Arthritis Research & Therapy 2023, 25: 10. PMID: 36670487, PMCID: PMC9854186, DOI: 10.1186/s13075-023-02990-3.Peer-Reviewed Original ResearchConceptsRaynaud's condition scoreCochin Hand Function ScaleSSc patientsClinic settingPatient-reported outcome instrumentsResultsNinety-two percentSystemic sclerosis patientsMann-Whitney U testHealthy control participantsCHF scoreSSc durationSSc-RPDigital ulcersCutaneous SScProvocation challengeRaynaud's phenomenonSclerosis patientsClinical trialsOutcome instrumentsOxygen saturationBurden assessmentPatientsCold provocationHemoglobin concentrationDisease severityLearnings from clinical trials in patients with connective tissue disease-associated interstitial lung disease
Higuero Sevilla J, Memon A, Hinchcliff M. Learnings from clinical trials in patients with connective tissue disease-associated interstitial lung disease. Arthritis Research & Therapy 2023, 25: 118. PMID: 37422652, PMCID: PMC10329300, DOI: 10.1186/s13075-023-03090-y.Peer-Reviewed Original ResearchConceptsConnective tissue disease-associated interstitial lung diseaseInterstitial lung diseaseTreatment of patientsMycophenolate mofetilSSc-ILDLung diseaseClinical trialsScleroderma Lung Study IIPatient-reported outcome instrumentsIdiopathic inflammatory myositisClinical trial resultsInflammatory myositisIntravenous cyclophosphamideOral cyclophosphamideSubcutaneous tocilizumabGood tolerabilityLung functionSystemic sclerosisTreatment armamentariumRheumatoid arthritisPatient outcomesSimilar efficacyOutcome instrumentsPatientsUS FoodCorrection: Learnings from clinical trials in patients with connective tissue disease‑associated interstitial lung disease
Higuero Sevilla J, Memon A, Hinchcliff M. Correction: Learnings from clinical trials in patients with connective tissue disease‑associated interstitial lung disease. Arthritis Research & Therapy 2023, 25: 132. PMID: 37501068, PMCID: PMC10373245, DOI: 10.1186/s13075-023-03123-6.Peer-Reviewed Original Research
2022
Epiregulin is a dendritic cell–derived EGFR ligand that maintains skin and lung fibrosis
Odell I, Steach H, Gauld S, Reinke-Breen L, Karman J, Carr T, Wetter J, Phillips L, Hinchcliff M, Flavell R. Epiregulin is a dendritic cell–derived EGFR ligand that maintains skin and lung fibrosis. Science Immunology 2022, 7: eabq6691. PMID: 36490328, PMCID: PMC9840167, DOI: 10.1126/sciimmunol.abq6691.Peer-Reviewed Original ResearchConceptsLung fibrosisDendritic cellsImmune cellsDiffuse cutaneous systemic sclerosisPersistence of fibrosisCutaneous systemic sclerosisExtent of fibrosisType I interferonSystemic sclerosisAutoimmune diseasesAntifibrotic targetsTherapeutic administrationMouse modelI interferonLung samplesLung explantsFibrosisFibrotic tissueImmune signalsEpiregulin expressionPatient's skinExtracellular matrix productionGenetic deficiencyEpiregulinEGFR ligands