Study Determines PAH-SYMPACT Suitable for Use Among Patients With SAPH

A new study aiming to understand sarcoidosis-associated pulmonary hypertension symptoms and their impacts on patients’ lives determined that the Pulmonary Arterial Hypertension-Symptoms and Impact questionnaire could be used to suitably assess patients.

A new study determined that the Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT) questionnaire is a suitable self-reporting instrument for assessing sarcoidosis-associated pulmonary hypertension (SAPH) symptoms and their impact on patients, according to findings published in BMC Pulmonary Medicine.

Patient-reported outcomes reported through the PAH-SYMPACT can be beneficial in understanding patients’ experience with SAPH and guiding disease management; currently no approved medicinal treatment for SAPH exists.

The PAH-SYMPACT assesses patients across 23 items: 11 on symptoms with 24-hour recall period, 11 on impacts with a 7-day recall period, and 1 item on oxygen use in the previous 24 hours. The symptom items are clustered into cardiopulmonary and cardiovascular domains, and the impact domains are clustered into physical and cognitive/emotional domains.

The study enrolled 11 patients diagnosed with SAPH, aged 18 to 85 years, who participated in qualitative 1-on-1 telephone interviews conducted in 2 parts: concept elicitation and cognitive debriefing. The concept elicitation portion of the interviews prompted participants to rate symptoms as “most bothersome or severe” and determine which impacts were “most difficult to cope with.”

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

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

Findings showed:

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

Although study findings indicate that the PAH-SYMPACT questionnaire is suitable for measuring symptoms and their impact in patients with SAPH, larger longitudinal studies are required to confirm the instrument’s suitability and 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 suitability of the pulmonary arterial hypertension-symptoms and impact (PAH-SYMPACT) questionnaire in patients with sarcoidosis-associated pulmonary hypertension (SAPH): a qualitative interview study. BMC Pulm Med. Published online November 12, 2021. doi:10.1186/s12890-021-01694-1