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BTS Clinical Statement on pulmonary sarcoidosis
  1. Muhunthan Thillai1,
  2. Christopher P Atkins2,
  3. Anjali Crawshaw3,
  4. Simon P Hart4,
  5. Ling-Pei Ho5,6,
  6. Vasileios Kouranos7,
  7. Karen Patterson8,
  8. Nicholas J Screaton9,
  9. Joanna Whight10,
  10. Athol U Wells7
  1. 1 Cambridge Interstitial Lung Disease Unit, Royal Papworth Hospital, Cambridge, Cambridgeshire, UK
  2. 2 Department of Respiratory Medicine, Norfolk and Norwich University Hospital, Norwich, Norfolk, UK
  3. 3 Interstitial Lung Disease Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham, Birmingham, UK
  4. 4 Respiratory Research Group, Hull York Medical School/University of Hull, Cottingham, UK
  5. 5 MRC Human Immunology Unit, Weatherall Institute of Molecular Medicine, Oxford, UK
  6. 6 Oxford Centre for Respiratory Medicine, Churchill Hospital, Oxford, UK
  7. 7 Interstitial Lung Disease Unit, Royal Brompton Hospital, London, London, UK
  8. 8 Department of Clinical and Experimental Medicine, Brighton and Sussex Medical School, Brighton, UK
  9. 9 Radiology, Papworth Hospital NHS Trust, Cambridge, UK
  10. 10 SarcoidosisUK, London, UK
  1. Correspondence to Dr Muhunthan Thillai, Royal Papworth Hospital, Cambridge CB2 0AY, Cambridgeshire, UK; muhunthan.thillai{at}nhs.net

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Introduction

This British Thoracic Society (BTS) Clinical Statement addresses the diagnosis, evaluation and management of pulmonary sarcoidosis, with each section summarised with key clinical practice points. In an era in which medical practice is increasingly determined by evidence-based guidelines, it must be acknowledged from the outset that current evidence in sarcoidosis, especially with regard to treatment, is weak. Thus, a number of the conclusions in this Statement are based on expert opinion and accumulated clinical experience.

The diagnosis of pulmonary sarcoidosis is often challenging, with ongoing evolution in clinician views on the need for a tissue diagnosis. Historically, a biopsy diagnosis was considered mandatory and we provide guidance on when to offer bronchoscopy and which bronchoscopic procedure to perform. We also stress that decisions made by individual patients to decline bronchoscopy, when there is a highly probable but not definite clinical diagnosis, should be supported in most cases, with careful subsequent monitoring. The document includes sections on the diagnosis of cardiac sarcoidosis and pulmonary hypertension as either disorder may present to respiratory physicians as ‘symptomatic pulmonary sarcoidosis’.

Traditional treatment algorithms and their reported application in the medical literature tend to be based on a ‘one size fits all’ approach and this has often led to over-treatment and major steroid-related comorbidity. However, a great many patients do not need to be treated: the broad indications for initiating therapy are (1) a high risk of mortality or disability due to major organ involvement; and (2) unacceptable loss of quality of life. In this statement, we focus on the management of pulmonary disease; the management of concurrent cardiac sarcoidosis or pulmonary hypertension requires referral to expert subspecialist teams.

Key pulmonary management considerations are discussed in this Statement. While higher dose treatment regimens may be required in high-risk disease, a highly flexible patient-centred approach is …

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Footnotes

  • MT and AUW are joint chair authors.

  • Contributors The following individuals were responsible for drafting the manuscript for submission to Thorax. MT, CPA, AC, SPH, L-PH, VK, KP, NJS, JW, AUW. MT and AUW were the authors responsible for final completion of the manuscript. All authors approved the final content of the submitted draft.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Disclaimer A Clinical Statement reflects the expert views of a group of specialists who are well versed on the topic concerned, and who carefully examine the available evidence in relation to their own clinical practice. A Clinical Statement does not involve a formal evidence review and is not developed in accordance with clinical practice guideline methodology. Clinical Statements are not intended as legal documents or a primary source of detailed technical information. Readers are encouraged to consider the information presented and reach their own conclusions.

  • Competing interests Declarations of interest were completed in line with the BTS Policy and are available from the BTS Office on request.

  • Patient consent for publication Not required.

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

  • Data availability statement There are no data in this work.

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