BTS clinical statement on pulmonary sarcoidosis

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 a) a high risk of mortality or disability due to major organ involvement; and b) 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 sub-specialist teams. Key pulmonary management considerations are discussed in this statement. Whilst higher dose treatment regimens may be required in high-risk disease, a highly flexible patient-centred approach is essential when treatment is introduced solely for quality of life reasons. In this context, sustained high-dose therapy is usually inappropriate. Patients should be asked to weigh-up treatment benefits against adverse effects before longer-term treatment decisions are made, with the danger of important comorbidities arising from long-term treatment kept carefully in mind. Monitoring must be tailored to specific goals. Fatigue, a highly prevalent and often disabling symptom in sarcoidosis, requires a systematic approach. Above all, we highlight the need for active patient involvement in decision-making and this, in turn, requires attention to clear communication, discussed in the final part of this statement. Finally, it is important to note that in a few areas of diagnosis and management where there was a non-unanimous consensus amongst the statement authors, this is clearly indicated. Scope The Statement covers diagnosis and management of pulmonary sarcoidosis. Reference is also made to diagnosis of cardiac sarcoidosis and pulmonary hypertension as either disorder may present to respiratory physicians as ‘symptomatic pulmonary sarcoidosis’. Other extra pulmonary sarcoidosis diagnoses are not covered by this Statement and it is recommended that specialists with specific sarcoidosis knowledge be consulted when the disease is present outside the chest. Importantly, it may be difficult to identify the true extent of organ involvement leading to underestimation of disease outside the chest.