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Meta-Analysis
. 2024 Apr 11;19(1):156.
doi: 10.1186/s13023-024-03152-7.

Diagnostic delay of sarcoidosis: an integrated systematic review

Tergel Namsrai  1 , Christine Phillips  2 , Anne Parkinson  1 , Dianne Gregory  1   3 , Elaine Kelly  1   3 , Matthew Cook  4 , Jane Desborough  5
Affiliations
Meta-Analysis

Diagnostic delay of sarcoidosis: an integrated systematic review

Tergel Namsrai et al. Orphanet J Rare Dis. .

Abstract

Background: Sarcoidosis is a chronic inflammatory granulomatous disease of unknown cause. Delays in diagnosis can result in disease progression and poorer outcomes for patients. Our aim was to review the current literature to determine the overall diagnostic delay of sarcoidosis, factors associated with diagnostic delay, and the experiences of people with sarcoidosis of diagnostic delay.

Methods: Three databases (PubMed/Medline, Scopus, and ProQuest) and grey literature sources were searched. Random effects inverse variance meta-analysis was used to pool mean diagnostic delay in all types of sarcoidosis subgroup analysis. Diagnostic delay was defined as the time from reported onset of symptoms to diagnosis of sarcoidosis.

Results: We identified 374 titles, of which 29 studies were included in the review, with an overall sample of 1531 (694 females, 837 males). The overall mean diagnostic delay in all types of sarcoidosis was 7.93 months (95% CI 1.21 to 14.64 months). Meta-aggregation of factors related to diagnostic delay in the included studies identified three categories: (1) the complex and rare features of sarcoidosis, (2) healthcare factors and (3) patient-centred factors. Meta-aggregation of outcomes reported in case studies revealed that the three most frequent outcomes associated with diagnostic delay were: (1) incorrect diagnosis, (2) incorrect treatment and (3) development of complications/disease progression. There was no significant difference in diagnostic delay between countries with gatekeeper health systems (where consumers are referred from a primary care clinician to specialist care) and countries with non-gatekeeper systems. No qualitative studies examining people's experiences of diagnostic delay were identified.

Conclusion: The mean diagnostic delay for sarcoidosis is almost 8 months, which has objective consequences for patient management. On the other hand, there is a paucity of evidence about the experience of diagnostic delay in sarcoidosis and factors related to this. Gaining an understanding of people's experiences while seeking a diagnosis of sarcoidosis is vital to gain insight into factors that may contribute to delays, and subsequently inform strategies, tools and training activities aimed at increasing clinician and public awareness about this rare condition.

Trial registration: PROSPERO Registration number: CRD42022307236.

Keywords: Diagnostic delay; Meta-aggregation; Meta-analysis; Misdiagnosis; Rare disease; Sarcoidosis; Systematic review.

PubMed Disclaimer

Conflict of interest statement

The authors have declared no conflict of interest.

Figures

Fig. 1
Fig. 1
Selection flow chart of studies included in the systematic review
Fig. 2
Fig. 2
Pooled mean diagnostic delay in sarcoidosis
Fig. 3
Fig. 3
Meta-aggregation results of factors related to diagnostic delay in sarcoidosis
Fig. 4
Fig. 4
Meta-aggregation of outcomes of diagnostic delay in case studies
See this image and copyright information in PMC

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