2018
Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?
Wolters PJ, Blackwell TS, Eickelberg O, Loyd JE, Kaminski N, Jenkins G, Maher TM, Molina-Molina M, Noble PW, Raghu G, Richeldi L, Schwarz MI, Selman M, Wuyts WA, Schwartz DA. Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? The Lancet Respiratory Medicine 2018, 6: 154-160. PMID: 29413083, PMCID: PMC5903445, DOI: 10.1016/s2213-2600(18)30007-9.Peer-Reviewed Original ResearchConceptsIdiopathic pulmonary fibrosisPulmonary fibrosisDevelopment of IPFFatal lung diseaseAlveolar epithelial cellsClinical presentationDistal airwaysLung diseaseFibroblast fociSubpleural fibrosisMicroscopic honeycombingPathogenic mechanismsFibrosisDiseaseEpithelial cellsMolecular mechanismsAirwayPathogenesisHoneycombingCentral driver
2015
Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis Study. Alpha-1 Protocol
Strange C, Senior RM, Sciurba F, O’Neal S, Morris A, Wisniewski SR, Bowler R, Hochheiser HS, Becich MJ, Zhang Y, Leader JK, Methé BA, Kaminski N, Sandhaus RA, Group* F. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis Study. Alpha-1 Protocol. Annals Of The American Thoracic Society 2015, 12: 1551-1560. PMID: 26153726, PMCID: PMC4627425, DOI: 10.1513/annalsats.201503-143oc.Peer-Reviewed Original ResearchMeSH KeywordsAdultAgedAged, 80 and overAlpha 1-Antitrypsin DeficiencyBronchoalveolar LavageCross-Sectional StudiesExercise ToleranceFemaleGenomicsGenotypeHumansMaleMicrobiotaMiddle AgedPhenotypeProspective StudiesPulmonary Disease, Chronic ObstructivePulmonary EmphysemaResearch DesignRespiratory Function TestsSarcoidosisTomography, X-Ray ComputedConceptsAlpha-1 antitrypsin deficiencyAugmentation therapyBronchoalveolar lavageAntitrypsin deficiencyClinical presentationPiZZ individualsAlpha-1-antitrypsin augmentation therapyAlpha-1 antitrypsin genotypeChronic obstructive pulmonary disease phenotypesPulmonary function testingAge 35 yearsVariable clinical presentationCross-sectional studyAlpha-1 antitrypsinIntermediate outcome measuresPulmonary disease phenotypesUnique genetic causeExercise capacityTherapeutic trialsChest tomographyClinical symptomsCOPD pathogenesisCOPD phenotypesFunction testingCOPD StudyUpdate in Diffuse Parenchymal Lung Disease 2013
Rosas IO, Kaminski N. Update in Diffuse Parenchymal Lung Disease 2013. American Journal Of Respiratory And Critical Care Medicine 2015, 191: 270-274. PMID: 25635490, PMCID: PMC4351573, DOI: 10.1164/rccm.201405-0856up.Peer-Reviewed Original ResearchMeSH KeywordsAnti-Inflammatory Agents, Non-SteroidalClinical Trials, Phase III as TopicDisease ProgressionDrug ApprovalEnzyme InhibitorsGenomicsHumansIdiopathic Pulmonary FibrosisIndolesLung Diseases, InterstitialPolymorphism, GeneticPyridonesTreatment OutcomeUnited StatesUnited States Food and Drug AdministrationConceptsMajority of patientsDistinct clinical presentationsSignificant clinical implicationsClinical presentationPulmonary fibrosis researchDisease progressionClinical implicationsGenetic biomarkersPatientsFibrosis researchGenetic variantsBiological mechanismsIPFNew biological mechanismsNintedanibPirfenidonePeriodHistoric approval