Abstract
BackgroundSarcoidosis-associated pulmonary hypertension (SAPH) is a prevalent and serious complication of sarcoidosis. No SAPH-specific self-report instruments for assessing SAPH symptoms and their impact on patients are available to date. This study sought to determine whether the Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT™) questionnaire is suitable for use in patients with SAPH.MethodsPatients diagnosed with SAPH participated in qualitative one-on-one telephone interviews to better understand SAPH symptoms and their impacts on patients’ lives and to determine the appropriateness of the PAH-SYMPACT™ for use in patients with SAPH. The interviews comprised concept elicitation, completion of the PAH-SYMPACT™, and cognitive debriefing. Interview transcripts were analyzed by content analysis.ResultsEleven patients with SAPH were interviewed between August 2019 and June 2020. In the concept elicitation, all 11 participants endorsed shortness of breath and nine participants (82%) rated it as their “most bothersome or severe” symptom. Impacts endorsed by all 11 participants were difficulty walking uphill or up stairs and difficulty in performing daily activities. Cognitive debriefing indicated that the PAH-SYMPACT™ items were relevant and understandable to most participants and reflected their experiences of SAPH. Participants indicated that no key symptoms or impacts of SAPH were missing. They also reported that the PAH-SYMPACT™ instructions and response options were clear, and that it would be feasible to complete the 11 symptom items and one oxygen use item as part of their daily schedule.ConclusionsThis study suggests the PAH-SYMPACT™ is suitable for assessing symptoms and their impact in patients with SAPH. However, larger longitudinal studies are needed to confirm that it is fit for use in this patient population and that it can be used to reliably detect temporal changes in patients’ symptom status.Trial registration Not applicable.
Highlights
Sarcoidosis-associated pulmonary hypertension (SAPH) is a prevalent and serious complication of sarcoidosis
This study suggests the pulmonary arterial hypertension (PAH)-SYMPACTTM is suitable for assessing symptoms and their impact in patients with SAPH
Most participants indicated that the symptom and impact items included in the PAHSYMPACTTM were relevant to their experiences of SAPH and sarcoidosis, even if they had not experienced them in the previous 24 h or 7 days
Summary
Sarcoidosis-associated pulmonary hypertension (SAPH) is a prevalent and serious complication of sarcoidosis. This study sought to determine whether the Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACTTM) questionnaire is suitable for use in patients with SAPH. Up to a fifth of patients with pulmonary sarcoidosis develop advanced disease characterized by pulmonary fibrosis [6]. An estimated 6–28% of patients with sarcoidosis [7,8,9] and up to 74% of patients with advanced pulmonary sarcoidosis suffer from pulmonary hypertension (PH) as a complication [1, 10, 11]. Sarcoidosis-associated PH (SAPH) causes significant morbidity and mortality over and above that caused by sarcoidosis itself [1, 11,12,13,14].
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