Study determines that PAH-SYMPACT is suitable for use in patients with SAPH

New study to understand pulmonary hypertension symptoms associated with sarcoidosis and their impact on the lives of patients determined that the Pulmonary Arterial Hypertension Symptom and Impact Questionnaire could be used to properly assess patients .

According to the findings published in BMC Pulmonary Medicine.

Patient-reported outcomes reported through PAH-SYMPACT may be beneficial in understanding patient experience with SAPH and guiding disease management; there is currently no approved drug treatment for SAPH.

PAH-SYMPACT assesses patients through 23 items: 11 on symptoms with a 24-hour booster period, 11 on impacts with a 7-day booster period and 1 item on oxygen consumption over 24 hours. previous hours. Symptom items are grouped into cardiopulmonary and cardiovascular domains, and impact domains are grouped into physical and cognitive / emotional domains.

The study included 11 patients diagnosed with SAPH, aged 18 to 85, who participated in individual qualitative telephone interviews conducted in 2 parts: concept elicitation and cognitive debriefing. The elicitation portion of the interview concept prompted participants to rank the symptoms as “most troublesome or most severe” and determine which impacts were “most difficult to manage”.

After the interviews, participants were asked to complete the PAH-SYMPACT questionnaire and assess the comprehensibility, relevance and clarity of the items listed. Participants were also asked to indicate the level of improvement they considered significant.

Investigators used transcripts of participants’ interviews to understand the symptoms of SAPH, their impacts on the lives of patients, and to determine the suitability of PAH-SYMPACT for use in patients with SAPH.

The results showed:

  • All 11 participants approved of shortness of breath and 9 participants (82%) rated it as their “most bothersome or serious” symptom.
  • All 11 participants reported having difficulty walking uphill or upstairs and difficulty performing daily activities
  • The cognitive debriefing indicated that the PAH-SYMPACT elements were relevant, understandable and reflected the experiences of most participants with SAPH
  • Participants reported that PAH-SYMPACT’s instructions and response options were clear and that it would be possible to complete all 11 symptom items and 1 daily oxygen uptake item.
  • In general, participants indicated that a one-point improvement was significant if starting from a score representing moderate symptoms or impacts.
  • A 2-3 point improvement was considered significant if it started from a score representing more severe symptoms or impacts

Although the study results indicate that the PAH-SYMPACT questionnaire is suitable for measuring symptoms and their impact in patients with SAPH, larger longitudinal studies are needed to confirm the suitability of the instrument and its ability to accurately detect symptom status among the patient population, the authors concluded.


Currie BM, Davies EW, Beaudet A, Stassek L, Kleinman L and Baughman RP. Symptoms, impacts and relevance of the questionnaire on symptoms and impact of pulmonary arterial hypertension (PAH-SYMPACT) in patients with pulmonary hypertension associated with sarcoidosis (SAPH): a qualitative interview study. BMC Pulm Med. Published online November 12, 2021. doi: 10.1186 / s12890-021-01694-1