Health-related quality of life in cardiac sarcoidosis: a systematic review

Abstract

People living with cardiac sarcoidosis (CS) are likely to have worse clinical outcomes and greater impairment on health-related quality of life (HRQoL) than other sarcoidosis manifestations. CS can result in a constellation of intrusive symptoms (such as palpitations, dizziness, syncope/pre-syncope, chest pain, dyspnoea, orthopnoea, or peripheral oedema) and/or life-threatening episodes, requiring consideration of invasive cardiac procedures for diagnosis and for the management of acute events. Additionally, the presence of multisystemic involvement and persistent non-specific sarcoidosis symptoms negatively affect HRQoL. A systematic review was undertaken to explore the impact of CS on HRQoL in adults with CS. Multiple bibliographic databases were searched for studies with HRQoL as primary or secondary outcomes in CS (PROSPERO registration: CRD42019119752). Data extraction and quality assessments were undertaken independently by two authors. From the initial 1609 identified records, only 11 studies included CS patients but none specifically reported HRQoL scores for CS patients. The average representation of CS patients was 14.5% within these cohorts (range 2-22%). The majority (73%) was conducted in single-centre tertiary care settings, and only one study (9%) included longitudinal HRQoL data. CS patients were among those sarcoidosis patients with impaired HRQoL and worse outcomes, requiring higher doses of sarcoidosis-specific therapy which contribute to further deterioration of HRQoL. Sarcoidosis studies do not incorporate stratified HRQoL scores for CS patients. While there is a need for longitudinal and multicentre studies assessing HRQoL outcomes in CS cohorts, the development of CS-specific tools is also needed.

Keywords: Cardiac sarcoidosis; Health-related quality of life (HRQoL); Patient-reported outcome measures (PROMS); Systematic review.

Graphical abstract

Figure 1

Adapted PRISMA flow diagram. The PRISMA diagram details our search and selection process applied during the overview. *The four ‘key papers’ included three peer-reviewed publications for the sarcoidosis-specific HRQoL questionnaires (SHQ, KSQ, and SAT) and one study which was selected for inclusion in the review (identified via electronic databases). **This supplementary searching technique included the following seven ‘key authors’ in HRQoL for sarcoidosis: Baughman, R.P.; Birring, S.S.; Drent, M.; Gozdenovic, B.S; Judson, M.A.; Wells, A.U.; and Wijsenbeek, M.S. AMED, allied and complementary medicine database; ASSIA, applied social sciences index and abstracts; BNI, British nursing index; CINAHL, cumulated index of nursing and allied health literature; CS, cardiac sarcoidosis; EThOS, electronic theses online service; HRQoL, health-related quality of life; JBI, Joanna Briggs Institute; KSQ, King’s sarcoidosis questionnaire; SAT, sarcoidosis assessment tool; SHQ, sarcoidosis health questionnaire.

Figure 2

An illustration of the quality assessment (CASP checklist for cohort studies) for the 11 included studies. CASP, Critical Appraisal Skills Programme.

Figure 3

Risk of bias across the studies (n = 11). Eight of the 11 studies provided limited or no information about the method of recruitment; we considered these studies as unclear risk of selection bias. Four of the studies did not provide sufficient information to determine if the outcome was measured accurately and were deemed as unclear for detection bias. Based on a review of evidence and determined a priori, significant factors were identified to assess confounding bias. Three out of the four studies with follow-up did not provide sufficient information to determine completeness rates and were therefore deemed as unclear risk of attrition bias. Considering that none of the studies fulfilled the full CASP checklist, all the studies were marked as unclear risk for other bias. Additionally, the included studies did not provide sufficient socio-demographic characteristics. For example, three studies did not report the ethnicity of their samples,^,, and only two studies provided data for the occupation and/or education level.^, Furthermore, the following reasons were noted to be deemed as unclear risk of other bias:

1. Limited CS representation (4–5%), socio-demographically imbalanced characteristics, scores for the SF-36 subscales not provided.^,

2. Limited CS representation (8%), no details were provided for the 25% of the sarcoidosis population who did not return study questionnaires.

3. Limited CS representation (3%), two references were used to describe the cohort of participants, but the provided samples were not matching with the reported data.

4. Limited CS representation (2%).

5. Online survey with no clinical characteristics. Self-reported socio-demographic characteristics. Only the SHQ total score was provided.

6. Retrospective analysis. Over half (363/660, 55%) of the cohort did not complete the SAT due to the validation date.

7. Monthly self-completed assessments using a new online platform for 6 months, and the study only reported baseline HRQoL scores.

8. Limited CS representation (7%). Only patients undergoing pulmonary function test during the clinical routine visits were eligible. No confidence intervals included.

9. Data analysed retrospectively from a multi-national database (n = 456). Participants with advanced sarcoidosis (n = 231) were followed up every 6 months for 3 years, but only baseline HRQoL assessments were reported. The analysis included over one-half (53%) of the cohort with advanced sarcoidosis and completed a 12-month visit.

10. Participants completed a follow-up 5-year visit, but HRQoL assessments were included only at baseline.

11. Other bias was deemed as unclear risk for all the studies considering that none of the studies fulfilled the full CASP checklist for cohort studies as detailed in Figure 2.