a Clinics of Respiratory Medicine, University Hospital Basel, Switzerland
b Division of Pulmonary Medicine, Cantonal Hospital St Gallen, Switzerland
We thank Carratù et al. for their technical comment  on our publication about the effect of bosentan in patients with steroid-resistant pulmonary sarcoidosis . The authors have shown previously that endothelin-1 (ET-1) urine levels were significantly higher in patients with active sarcoidosis as compared with healthy controls . Furthermore, urine ET-1 levels decreased after prednisone treatment, which was paralleled by an improved clinical status . Based on this observation the authors suggest measuring urine ET-1 levels before and after bosentan treatment, as this might be a prognostic factor.
The identification of specifically targetable traits in a complex disorder like sarcoidosis is of paramount importance. However, we would like to emphasise that the role of ET-1 urine levels in patients with sarcoidosis has not been elucidated yet and thus it is rather speculative to propose it as a prognostic tool. Neither has it been validated as a predictive tool. Moreover, based on our data  and on current knowledge there is no role for bosentan in the treatment of patients with steroid-resistant pulmonary sarcoidosis and thus the effect of bosentan on urine ET-1 levels in sarcoidosis patients does not seem of priority interest.
No financial support and no other potential conflict of interest relevant to this article was reported.
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Katrin E. Hostettler, MD, PhD, University Hospital Basel, Petersgraben 4, CH-4031 Basel, katrin.hostettler[at]usb.ch
1 Carratù P, Dragonieri S, Resta O. Comment on: Hostettler K, et al. Bosentan for patients with steroid-resistant pulmonary sarcoidosis: a randomised controlled trial. Swiss Med Wkly. 2019;149:w20132. doi: http://dx.doi.org/10.4414/smw.2019.20132
2 Hostettler K, Baty F, Kleiner R, Junker L, Tamm M, Brutsche M. Bosentan for patients with steroid-resistant pulmonary sarcoidosis: a randomised controlled trial. Swiss Med Wkly. 2018;148:w14677. doi:. http://dx.doi.org/10.4414/smw.2018.14677 PubMed
3 Sofia M, Mormile M, Faraone S, Alifano M, Carratù P, Carratù L. Endothelin-1 excretion in urine in active pulmonary sarcoidosis and in other interstitial lung diseases. Sarcoidosis. 1995;12(2):118–23. PubMed
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