Sarcoidosis in the UK: insights from British Thoracic Society registry data

Abstract

IntroductionThe British Thoracic Society Sarcoidosis Registry allows physicians to record clinical data after gaining written consent from patients. The registry’s aim is to phenotype sarcoidosis in the UK.MethodsBetween February 2013 and July 2017, demographic details for 308 patients (with complete clinical data for 205 patients) presenting to 24 UK hospitals were recorded. This data was analysed to detail methods of presentation, diagnosis and management.ResultsFatigue was a significant complaint, affecting 30% of all patients. The most prevalent CT findings were nodules (in 77% of cases) with traction bronchiectasis (11%), distortion (9%) and ground glass (5%) less prominent. Of 205 patients with complete clinical data, only 64% had a diagnostic tissue biopsy. 35% of all patients underwent endobronchial ultrasound-guided transbronchial needle aspirate (EBUS-TBNA) with 15% having a transbronchial biopsy. Use of EBUS-TBNA showed an overall increase over time, from 28% of all patients in 2013 to 43% in 2016. The most common steroid sparing treatment was methotrexate, but 42% of patients were not initiated on any pharmacological treatment at the time of inclusion.DiscussionFatigue was common and has shown association with poor quality of life. We therefore suggest using a fatigue questionnaire as part of all new patient assessments. It may be that EBUS-TBNA should be reserved for cases of stage I or II disease where there is a reported higher yield than using transbronchial biopsy alone. Bronchoalveolar lavage was not widely used in our data, but it is generally a safe and useful adjunct and should be used more widely.

Sarcoidosis diagnosis and treatment in Sweden: A register-based assessment of variations by region and calendar period

“…Four in ten patients with sarcoidosis received at least one prescription of systemic corticosteroids around the time of diagnosis, whereas second line (steroid-sparing) treatments were rarely prescribed at diagnosis. These observations are consistent with reports from the United States, the United Kingdom, Spain, and Poland [19][20][21][22][23][24].…”

BTS Clinical Statement on pulmonary sarcoidosis

“…Analysis of the British Thoracic Society Sarcoidosis Registry showed that 8% of all patients with pulmonary sarcoidosis were prescribed inhaled steroids (94). Although an obstructive defect is present in almost a quarter of UK patients with sarcoidosis (95), there is no evidence for inhaler efficacy (96).…”

Australasian interstitial lung disease registry (AILDR): objectives, design and rationale of a bi-national prospective database

“…2000) Not published Not published United States RAPID (NCT03297775) June 2017 - current RA and ILD inc. IPF (Est. 500) Not published Not published International EUSTAR [ 27 ] June 2004 - current SSc inc. SSc-ILD (> 15,000) 92.2 (±21.3) 68.3 (±21.1) Australia SASR [ 28 ] 1993–2007 Scleroderma (786) Not published Not published UK BRILL [ 29 ] 1987 - current RA and ILD (230) 101 (54–145) b 70 (44–117) c 61 (33–106) b 52 (22–109) c UK BTS [ 30 ] Feb 2013 - current Sarcoidosis (> 300) 97 78.2 Europe eurIPFreg (NCT02951416) Sept 2009 - current ILDs inc. IPF (> 1080) Not published Not published Abbreviations : AILDR Australasian ILD registry, CARE-PF The Canadian Registry for Pulmonary Fibrosis, PFF-PR Pulmonary Fibrosis Foundation Patient Registry, REGIS Romanian Registry for Interstitial Lung Diseases, UIP Usual Interstitial Pneumonia, RIPID Registry of Diffuse Infiltrative Pulmonary Diseases, RAPID Rheumatoid Arthritis patients at Risk for ILD, RA Rheumatoid Arthritis, EUSTAR European Scleroderma Trials and Research Group, SSc Systemic Sclerosis, SASR South Australia Scleroderma Register, BRILL British Rheumatoid Interstitial Lung network, BTS British Thoracic Society a Trial identifier where available b Limited disease on CT c Extensive disease on CT …”