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Clinical phenotyping: role in treatment decisions in sarcoidosis

Robert P. Baughman, Mary Beth Scholand, Franck F. Rahaghi
European Respiratory Review 2020 29: 190145; DOI: 10.1183/16000617.0145-2019
Robert P. Baughman
1University of Cincinnati Medical Center, Cincinnati, OH, USA
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  • For correspondence: bob.baughman@uc.edu
Mary Beth Scholand
2University of Utah Medical Center, Salt Lake City, UT, USA
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Franck F. Rahaghi
3Cleveland Clinic Florida, Weston, FL, USA
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  • FIGURE 1
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    FIGURE 1

    Delphi consensus on whether patients should be treated based on the presence of pulmonary symptoms, pulmonary function testing and imaging. Bold indicates consensus. HRCT: high-resolution computed tomography; PFT: pulmonary function test; FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide.

  • FIGURE 2
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    FIGURE 2

    Delphi consensus on the role of extrapulmonary disease in treatment decisions. Bold indicates consensus.

  • FIGURE 3
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    FIGURE 3

    Delphi consensus on clinical situations in which immediate therapy is warranted. PFT: pulmonary function test; DLCO: diffusing capacity of the lung for carbon monoxide; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s.

  • FIGURE 4
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    FIGURE 4

    Delphi consensus on clinical situations in which second-line therapies (usually non-biologic cytotoxic agents) and third-line therapies (usually biologic agents) should be considered. Bold indicates consensus.

Tables

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  • TABLE 1

    Phenotype scales reported in sarcoidosis

    First author [ref.]PhenotypeDescription of developing phenotypePatients nValidationSingle versus multi-centreEstablished association to genotypeComments
    Scadding [5]PulmonaryChest radiograph stage136NoSingleNoDid not use computed tomography
    Neville [7]Acute versus chronicRate of resolution818NoSingleNoDid not account for more than one factor in patient
    Grunewald [3, 8]Löfgren versus non-LöfgrenClinical outcome of Löfgren patients754YesSingleYesSeries of studies, may not apply to other racial groups
    Wasfi [9]Severe versus non-severeBackward regression based on clinical parameters104Two different groups of clinicians assessed same patientsSingleNoBased on expert opinion, no follow-up analysis
    Prasse [10]Acute versus chronic and duration of treatmentEvaluated patients seen within 1 year of presentation225NoSingleNoMore useful for acute disease
    Baughman[11]Acute versus chronic including therapyDeveloped criteria for long-term outcome500NoMulti-centreNoRetrospective look based on expert opinion
    Rodrigues[12]Acute, relapse, fibrosisFactor analysis137NoMulti-centreNoPhenotypes were not distinct
    Walsh [13]Severe versus non-severeRegression analysis of multiple factors251Yes (additional 252)SingleNoOnly focused on advanced pulmonary disease
    Moller [14]Various groups of patientsExpert opinion of grouping of patientsNot givenNoMulti-centreYesEstablished criteria to be studied
    Schupp [15]Organ clusteringMulti-factor analysis based on gene expression2163NoMulti-centreYesUsed genetic profile to identify associated organ manifestations

Supplementary Materials

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    Supplementary_appendix ERR-0145-2019_Supplementary_appendix

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Clinical phenotyping: role in treatment decisions in sarcoidosis
Robert P. Baughman, Mary Beth Scholand, Franck F. Rahaghi
European Respiratory Review Mar 2020, 29 (155) 190145; DOI: 10.1183/16000617.0145-2019

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Clinical phenotyping: role in treatment decisions in sarcoidosis
Robert P. Baughman, Mary Beth Scholand, Franck F. Rahaghi
European Respiratory Review Mar 2020, 29 (155) 190145; DOI: 10.1183/16000617.0145-2019
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  • Article
    • Abstract
    • Abstract
    • Introduction
    • History of clinical phenotyping in sarcoidosis
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