TY - JOUR
T1 - Diagnostic delay of myositis
T2 - an integrated systematic review
AU - Namsrai, Tergel
AU - Parkinson, Anne
AU - Chalmers, Anita
AU - Lowe, Christine
AU - Cook, Matthew
AU - Phillips, Christine
AU - Desborough, Jane
N1 - Publisher Copyright:
© 2022, The Author(s).
PY - 2022/12
Y1 - 2022/12
N2 - Background: Idiopathic inflammatory myopathies (IIM) are a heterogenous group of rare muscular autoimmune diseases characterised by skeletal muscle inflammation with possible diagnostic delay. Our aim was to review the existing evidence to identify overall diagnostic delay for IIM, factors associated with diagnostic delay, and people’s experiences of diagnostic delay. Methods: Three databases and grey literature sources were searched. Diagnostic delay was defined as the period between the onset of symptoms and the year of first diagnosis of IIM. We pooled the mean delay using random effects inverse variance meta-analysis and performed subgroup analyses. Results: 328 titles were identified from which 27 studies were included. Overall mean diagnostic delay was 27.91 months (95% CI 15.03–40.79, I2 = 99%). Subgroup analyses revealed a difference in diagnostic delay between non-inclusion body myositis (IBM) and IBM types. There was no difference in diagnostic delay between studies in which myositis specific autoantibodies (MSA) were tested or not tested. In countries with gatekeeper health systems, where primary care clinicians authorize access to specialty care, people experienced longer periods of diagnostic delay than people with IIM in countries with non-gatekeeper systems. While studies discussed factors that may influence diagnostic delay, significant associations were not identified. No qualitative studies examining people’s experiences of diagnostic delay were identified. Conclusion: Diagnostic delay of IIM has extensive impacts on the quality of life of people living with this disease. Understanding the experiences of people with IIM, from symptom onset to diagnosis, and factors that influence diagnostic delay is critical to inform clinical practice and training activities aimed at increasing awareness of this rare disease and expediting diagnosis. Trial registration: PROSPERO Registration number: CRD42022307236 URL of the PROSPERO registration: https://www.crd.york.ac.uk/PROSPEROFILES/307236_PROTOCOL_20220127.pdf
AB - Background: Idiopathic inflammatory myopathies (IIM) are a heterogenous group of rare muscular autoimmune diseases characterised by skeletal muscle inflammation with possible diagnostic delay. Our aim was to review the existing evidence to identify overall diagnostic delay for IIM, factors associated with diagnostic delay, and people’s experiences of diagnostic delay. Methods: Three databases and grey literature sources were searched. Diagnostic delay was defined as the period between the onset of symptoms and the year of first diagnosis of IIM. We pooled the mean delay using random effects inverse variance meta-analysis and performed subgroup analyses. Results: 328 titles were identified from which 27 studies were included. Overall mean diagnostic delay was 27.91 months (95% CI 15.03–40.79, I2 = 99%). Subgroup analyses revealed a difference in diagnostic delay between non-inclusion body myositis (IBM) and IBM types. There was no difference in diagnostic delay between studies in which myositis specific autoantibodies (MSA) were tested or not tested. In countries with gatekeeper health systems, where primary care clinicians authorize access to specialty care, people experienced longer periods of diagnostic delay than people with IIM in countries with non-gatekeeper systems. While studies discussed factors that may influence diagnostic delay, significant associations were not identified. No qualitative studies examining people’s experiences of diagnostic delay were identified. Conclusion: Diagnostic delay of IIM has extensive impacts on the quality of life of people living with this disease. Understanding the experiences of people with IIM, from symptom onset to diagnosis, and factors that influence diagnostic delay is critical to inform clinical practice and training activities aimed at increasing awareness of this rare disease and expediting diagnosis. Trial registration: PROSPERO Registration number: CRD42022307236 URL of the PROSPERO registration: https://www.crd.york.ac.uk/PROSPEROFILES/307236_PROTOCOL_20220127.pdf
KW - Diagnostic delay
KW - Idiopathic inflammatory myositis
KW - Meta-analysis
KW - Myositis
KW - Systematic review
UR - http://www.scopus.com/inward/record.url?scp=85142270393&partnerID=8YFLogxK
U2 - 10.1186/s13023-022-02570-9
DO - 10.1186/s13023-022-02570-9
M3 - Review article
SN - 1750-1172
VL - 17
JO - Orphanet Journal of Rare Diseases
JF - Orphanet Journal of Rare Diseases
IS - 1
M1 - 420
ER -