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Outcomes of definite vs probable/presumed cardiac sarcoidosis: a systematic review and meta-analysis
Raheel Ahmed1, Areeba Ahsan2, Mushood Ahmed3
1Royal Brompton Hospital, part of Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; National Heart and Lung Institute, Imperial College London, United Kingdom.
Insights
Diagnosing cardiac sarcoidosis (CS) is challenging. This study found no significant outcome differences between definite CS (with biopsy) and probable CS (without biopsy), suggesting histological confirmation isn't always necessary.
Area of Science:
- Cardiology
- Immunology
- Pulmonology
Background:
- Cardiac sarcoidosis (CS) diagnosis is complex, often requiring endomyocardial biopsy (EMB) per 2014 Heart Rhythm Society guidelines.
- Definite CS diagnosis relies on EMB, while probable/presumed CS uses clinical and imaging criteria.
Purpose of the Study:
- To compare clinical outcomes between patients with definite CS and those with probable/presumed CS.
- To evaluate if histological confirmation impacts patient prognosis in cardiac sarcoidosis.
Main Methods:
- A systematic meta-analysis of studies published after 2014 from PubMed/MEDLINE, Embase, and Cochrane Library.
- Risk ratios (RR) and 95% confidence intervals (CI) were calculated using a random effects model.
Main Results:
- Six studies comprising 2,204 patients were analyzed, with a median follow-up of 40.5 months.
- No statistically significant difference in the composite endpoint (RR: 1.80, 95% CI: 0.93 to 3.49) or all-cause death (RR: 1.01, 95% CI: 0.48 to 2.10) between definite and probable/presumed CS.
- The mean age of the cohort was 56.8 years (±13.6 years).
Conclusions:
- Definite and probable/presumed cardiac sarcoidosis show equivalent clinical courses and prognoses.
- This supports a multidisciplinary approach to CS management, indicating histological confirmation is not essential for diagnosis or treatment.
Background:
Diagnosing cardiac sarcoidosis (CS), which can be associated with arrhythmias and heart failure, remains challenging despite multiple advances over time. The 2014 Heart Rhythm Society (HRS) consensus statement recommends an endomyocardial biopsy (EMB) to establish a definite diagnosis of CS. In the absence of a positive EMB, a diagnosis of probable or presumed CS is made on the basis of clinical and imaging criteria.
Objective:
To investigate whether there is any difference in outcomes between definite vs probable/presumed CS.
Methods:
PubMed/MEDLINE, Embase, and the Cochrane Library databases were searched for relevant studies published after 2014. Risk ratios (RR) with 95% confidence intervals (CI) were calculated using the random effects model and presented in forest plots.
Results:
6 studies involving 2,204 patients were identified. The cohort had a mean age of 56.8 years (SD: ±13.6 years). The median duration of follow-up was 40.5 months. No statistically significant difference was observed between definite and probable/presumed CS for reduced risk of the composite endpoint (RR: 1.80, 95% CI: 0.93 to 3.49), and all-cause death (RR: 1.01, 95% CI: 0.48 to 2.10).
Conclusion:
This meta-analysis demonstrated the equivalence of clinical course and prognosis between definite and probable/presumed CS. This highlights the importance of a multi-disciplinary approach to CS care and emphasizes that histological confirmation should not be a prerequisite to diagnose or manage this condition.
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