Background Pulmonary hypertension (PH) has emerged as a critical cause of death in systemic sclerosis (SSc). Recent data have highlighted the poorer outcomes of SSc associated PAH as compared to the idiopathic forms. Therefore, the management of SSc patients at risk of PAH risk must be improved to allow early diagnosis. However, there is presently no guideline regarding the parameters that should lead the physician to perform right heart catheterization (RHC), the only tool unequivocally establishing the correct diagnosis.
Objectives By consensus, to identify the most appropriate indications for RHC in patients with SSc.
Methods A three-stage Delphi exercise involving worldwide PH experts (rheumatologists, pulmonologists and cardiologists) was designed to answer the following question: “based on which parameters, performed on the basis of an annual screening of SSc patients in clinical practice, do you decide to refer patients for RHC?”. The Delphi exercise was performed between March 2011 and December 2011. The aim of the first stage was to obtain a comprehensive list of domains and tools to be considered before referring a SSc patient for RHC. This list combined evidence-based indications extracted from published reports on SSc-PH and expert opinions. For the second stage, experts were asked to rate each item proposed in the list, using a 5-point scale (1 indicates “not important/appropriate atall” and 5 indicates “very important/appropriate”). For the third stage, experts were asked to rate the items accepted after the second round, using the same 5-point scale. After stages 2 and 3, the number of domains and tools was reduced according to a cluster analysis.
Results 77 experts were contacted by e-mail to participate in this Delphi procedure. 47 (61%) participated in stage 1, 50 (65% of the 77) in stage 2, and 48 (62% of the 77) in stage 3. The list obtained after the first stage consisted on 7 domains (clinical, biomarkers, pulmonary function tests, echocardiography, cardiopulmonary exercise, imaging and EKG) containing a total of 142 tools. Cluster analysis performed after the second stage allowed discarding of 63 of the 142 initial tools. Cluster analysis performed after stage 3 reduced the EPOSS instrument to 3 domains containing 8 items (see table).
Conclusions Among experts in PH-SSc, a core set of indications for clinical practice has been defined to refer SSc patients to RHC in case of PH suspicion.This EPOSS instrument is the first expert guidelines for early PH detection that is based on validated consensus methods. Although these indications are recommended by this expert group, it is an interim tool. It will be necessary to formally validate and extend the EPOSS instrument in further studies.
Disclosure of Interest J. Avouac Grant/Research support from: Actelion, Pfizer, Speakers Bureau: Actelion, D. Huscher: None Declared, D. Furst: None Declared, O. Distler Grant/Research support from: Actelion, Pfizer, Ergonex, BMS, Sanofi-Aventis, United BioSource Corporation, medac, 4D Science, Boehringer-Ingelheim, Active Biotech and Roche in the area of potential treatments of scleroderma and its complications., Speakers Bureau: Actelion, Pfizer, Encysive and Ergonex, Y. Allanore Grant/Research support from: Actelion, Pfizer, Speakers Bureau: Actelion