STEPS study

Sarcoidosis is a granulomatous disorder of unknown cause. Current therapy is immunosuppressive, not curative and often ineffective, as we do not thoroughly understand the underlying pathogenesis. Oral corticosteroids are the first line of therapy in pulmonary sarcoidosis patients with significant pulmonary symptoms and/or progressive disease as determined by radiology or lung function. In the latter case, treatment is primarily aimed at preventing organ damage, although previous studies did not conclusively demonstrate a beneficial effect in preventing disease progression or pulmonary fibrosis. Nevertheless, current treatment guidelines advocate relatively long and high initial treatment regimes, without clear evidence for the optimal dose and duration of treatment. Importantly, prolonged treatment with prednisone is known to induce considerable side-effects/co-morbidity.
Several expert-opinions suggest a lower initial dose and shorter initial phase, based on retrospective case series. However, there is considerable variety in the response to treatment between sarcoidosis individuals and immunological mechanisms underlying this phenomenon have not been elucidated yet. These data suggest that treatment of sarcoidosis patients should be individualized, pursuing the lowest prednisone dose for the shortest period possible. However, prospective data on the early response towards prednisone treatment and tapering is lacking in a well-characterized cohort of pulmonary sarcoidosis patients.
Against this background, we aim to describe the pulmonary response to corticosteroid therapy and tapering in a cohort of newly treated pulmonary sarcoidosis patients, using a hand-held spirometer. With results of this study, in the future, we hope to individualize the treatment and tapering strategy for sarcoidosis patients. This could lead to prednisone dose sparing, reduction in co-morbidity and an increased quality of life of sarcoidosis patients. This treatment strategy has already been performed successfully in other chronic pulmonary diseases requiring prednisone treatment, like asthma.

Sarcoidosis is a granulomatous disorder of unknown cause. Current therapy is immunosuppressive, not curative and often ineffective, as we do not thoroughly understand the underlying pathogenesis. Oral corticosteroids are the first line of therapy in pulmonary sarcoidosis patients with significant pulmonary symptoms and/or progressive disease as determined by radiology or lung function. In the latter case, treatment is primarily aimed at preventing organ damage, although previous studies did not conclusively demonstrate a beneficial effect in preventing disease progression or pulmonary fibrosis. Nevertheless, current treatment guidelines advocate relatively long and high initial treatment regimes, without clear evidence for the optimal dose and duration of treatment. Importantly, prolonged treatment with prednisone is known to induce considerable side-effects/co-morbidity.



Several expert-opinions suggest a lower initial dose and shorter initial phase, based on retrospective case series. However, there is considerable variety in the response to treatment between sarcoidosis individuals and immunological mechanisms underlying this phenomenon have not been elucidated yet. These data suggest that treatment of sarcoidosis patients should be individualized, pursuing the lowest prednisone dose for the shortest period possible. However, prospective data on the early response towards prednisone treatment and tapering is lacking in a well-characterized cohort of pulmonary sarcoidosis patients.




Against this background, we aim to describe the pulmonary response to corticosteroid therapy and tapering in a cohort of newly treated pulmonary sarcoidosis patients, using a hand-held spirometer. With results of this study, in the future, we hope to individualize the treatment and tapering strategy for sarcoidosis patients. This could lead to prednisone dose sparing, reduction in co-morbidity and an increased quality of life of sarcoidosis patients. This treatment strategy has already been performed successfully in other chronic pulmonary diseases requiring prednisone treatment, like asthma.

Baseline visit, months 1,3,6,9,12

Patients participating in this study will be treated according to current guidelines. As such, diagnostic procedures or treatment will not be postponed during participation. Standard procedures will be executed during regular visits at the outpatient clinic. In course of the research:


1. Daily home monitoring of the pulmonary function (PF) will be performed with a hand-held spirometer (MicroDiary, CareFusion) during the first three months of treatment;


2. The patient is asked to keep record of his/her symptomatic response to therapy by filling out a weekly dyspnoea score (MRC scale) and a fatigue score (FAS score) during the first three months of treatment at home;

3. During the regular visits at the outpatient clinic, patient reported response to therapy will be objectified by two standardized questionnaires (SGRQ, SF-36, KSQ), in-hospital pulmonary function tests at the department of pulmonology, a chest X-ray, thorax-HRCT (only if physician’s choice), weight and laboratory data will be recorded;


4. The patient will be asked to donate an additional 60 ml peripheral blood for immunological analysis at the baseline visit, month 1,3 and 12 (i.e. amount of macrophages, NK-cells, dendritic cells, T-cells, B-cells and cytokines; apoptotic susceptibility towards FasL, IL-2 deprivation and prednisone; expression of activation, differentiation and apoptotic markers; immunosuppressive function of lymphocyte subsets and HLA-type will be examined). Blood will be collected during routine venapunction.