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Original research
Pulmonary sarcoidosis: differences in lung function change over time
  1. Michelle Sharp1,
  2. Kevin J Psoter2,
  3. Ali M Mustafa1,
  4. Edward S Chen1,
  5. Nancy W Lin1,
  6. Stephen C Mathai1,
  7. Nisha A Gilotra3,
  8. Michelle N Eakin1,
  9. Robert A Wise1,
  10. David R Moller1,
  11. Meredith C McCormack1
  1. 1Medicine, Division of Pulmonary and Critical Care Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland, USA
  2. 2Pediatrics, Division of General Pediatrics, Johns Hopkins School of Medicine, Baltimore, Maryland, USA
  3. 3Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
  1. Correspondence to Dr Michelle Sharp, Medicine, Johns Hopkins School of Medicine, Baltimore, MD 21224, USA; msharp5{at}jh.edu

Abstract

Introduction Given the heterogeneity of sarcoidosis, predicting disease course of patients remains a challenge. Our aim was to determine whether the 3-year change in pulmonary function differed between pulmonary function phenotypes and whether there were differential longitudinal changes by race and sex.

Methods We identified individuals seen between 2005 and 2015 with a confirmed diagnosis of sarcoidosis who had at least two pulmonary function test measurements within 3 years of entry into the cohort. For each individual, spirometry, diffusion capacity, Charlson Comorbidity Index, sarcoidosis organ involvement, diagnosis duration, tobacco use, race, sex, age and medications were recorded. We compared changes in pulmonary function by type of pulmonary function phenotype and for demographic groups.

Results Of 291 individuals, 59% (173) were female and 54% (156) were black. Individuals with restrictive pulmonary function phenotype had significantly greater 3-year rate of decline of FVC% (forced vital capacity) predicted and FEV1% (forced expiratory volume in 1 s) predicted course when compared with normal phenotype. We identified a subset of individuals in the cohort, highest decliners, who had a median 3-year FVC decline of 156 mL. Black individuals had worse pulmonary function at entry into the cohort measured by FVC% predicted, FEV1% predicted and diffusing capacity for carbon monoxide % predicted compared with white individuals. Black individuals’ pulmonary function remained stable or declined over time, whereas white individuals’ pulmonary function improved over time. There were no sex differences in rate of change in any pulmonary function parameters.

Summary We found significant differences in 3-year change in pulmonary function among pulmonary function phenotypes and races, but no difference between sexes.

  • Sarcoidosis
  • Rare lung diseases

Data availability statement

All data relevant to the study are included in the article or uploaded as supplemental information.

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Data availability statement

All data relevant to the study are included in the article or uploaded as supplemental information.

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Footnotes

  • Contributors MS had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis and is the guarantor of the manuscript. MS, KJP, AMM, ESC, NWL, SCM, NAG, MNE, RAW, DRM and MCM contributed substantially to the study design, data analysis and interpretation, and/or the writing of the manuscript.

  • Funding Research reported in this publication was supported by the National Heart, Lung, and Blood Institute of the National Institutes of Health under award numbers T32HL007534, K23HL163313 and K23HL148527.

  • Disclaimer The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.

  • Competing interests Research reported in this publication was supported by the National Heart, Lung, and Blood Institute of the National Institutes of Health to support AMM, NWL and MS, respectively. MS has been a co-investigator on grants with aTyr Pharma, Kinevant and Novartis without support. ESC has received grant support from aTyr Pharma, Kinevant and Novartis that does not pertain to the current manuscript. NAG has consulted for Kiniksa Pharmaceutical. SCM has consulted for Janssen, United Therapeutics and Merck. RAW has received grant funding from Sanofi, Chiesi and AstraZeneca, and has consulted for Galderma, AbbVie, AstraZeneca, Boehringer-Ingelheim, Contrafect, Savara, Kamada, Bristol Myers Squibb, Pulmonx, Scene Healthcare, Beyond Air and Puretech. DRM receives royalties from Taylor & Francis Group and is chairman and chief technical officer for Sarcoidosis Diagnostic Testing. MCM has received royalties from UpToDate for authorship and editorial work and has consulting relationships with Aridis, GlaxoSmithKline, NDD Medical Technologies, MCG Diagnostics and Boehringer Ingelheim.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.