Pulmonary sarcoidosis

Value-based healthcare in pulmonary sarcoidosis

Despite advances since sarcoidosis was first described, much remains unknown regarding the aetiology of the disease. Due to the heterogeneous clinical manifestation, treatment can vary from no treatment to thorough follow-up with a variety of medications. The appropriatetreatment for pulmonary sarcoidosis patients has not been well defined (Costabel et al. 1999). As stated in the Sarcoidosis Statement Committee, the symptoms that need corticosteroid therapy remain controversial. In order to tailor treatment well to the individual level, the availability of centre-specific outcome data has the potential to provide important advantages for quality improvement efforts.


The standard outcome set was designed specifically for pulmonary sarcoidosis patients. In order to be able to compare treatment outcomes among hospitals, not only the outcome measure and initial conditions need to be defined, but also a clear and uniform definition of the patient population itself is needed. The patient group is defined in line with the joint statement on sarcoidosis (Costabel et al. 1999). The international team acknowledges sarcoidosis is a very heterogeneous population. Therefore, it was decided there has to be pulmonary involvement, as 90 percent of sarcoidosis patients show pulmonary involvement. Specifically, the patient has to be: 1) Diagnosed with pulmonary sarcoidosis. 2) The diagnosis has to be performed by a pulmonologist (based on the joint statement on sarcoidosis) and 3) Extra-pulmonary sarcoidosis is not excluded.


The pulmonologists from the six centres of expertise worked from early 2014 to early 2015 on the definition of an international standard set of outcomes measures for patients with pulmonary sarcoidosis. The team convened in a combination of face-to-face meetings, webinars and surveys. A systematic approach was used based on the principles of value-based healthcare (Porter 2010) and the methodology introduced in heart care by Meetbaar Beter (Meetbaar Beter 2013). First, a detailed description of the care delivery process for pulmonary sarcoidosis patients was created, providing an overview of ‘value’ created by all specialties involved throughout the entire care chain. Combined with a literature review, this was used to create a list of all potentially relevant health outcomes for pulmonary sarcoidosis patients. In order to end up with a concise and feasible set of outcome measures, the most important outcomes were prioritised anonymously by all team members based on three criteria: impact of the outcome on quality of life, impact of quality of care on the outcome and the number of patients affected by the outcome. As a result, the standard set combines both clinical outcomes, such as mortality, symptoms and side effects, with patient-reported outcomes, such as quality of life. Discussions were organised to reach consensus on the final outcome set. Following a similar process, the most relevant initial conditions were selected in order to allow corrections for the complexity of the population.


Based on a retrospective patient cohort (n=533) and the literature (Broos et al., 2017) we have changed our Prednisone protocol. In our multi-centre data we found that the patients (n=123)  using Prednisone for a long period (>1 year) had a mean BMI at their first visit of 28,1 (SD 5,7) in Leuven University hospital, 31,2 (SD 7,5) in Cincinnati MC, 29,4 (SD 4,5) in Cleveland Clinic, 29,4 (SD 4,4) in the Erasmus MC, 28,2 (SD 7,8) in the Royal Brompton Hospital and 28,2 (SD 6,3) in the St. Antonius Hospital. In addition, of these 123 patients, 97 were already using Prednisone prior to visiting the clinic first time, which can possibly explain the high BMI’s.


Our new protocol on Prednisone was introduced at the St. Antonius hospital only, on the 1st of September 2017. We have also shared this protocol with the top-10 referring hospitals. With this new protocol, patients do not start on Prednisone when their BMI is higher than 25. In addition, patients will switch to 2nd line therapy when there is a weight gain of> 5% of their initial weight. We aim to minimize weight gain and therefore reduce harmful side-effects due to treatment with Prednisone.