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Diagnostic criteria for systemic lupus erythematosus: has the time come?

Abstract

Systemic lupus erythematosus (SLE) is a multiorgan disease with protean manifestations. Because SLE is uncommon and heterogeneous, its diagnosis can pose a considerable challenge, especially for clinicians with limited expertise of the disease. This is particularly true at the early stages of SLE, when an inadequate number of features to secure the diagnosis might be present, and for patients presenting with uncommon features, which can nonetheless be severe and require prompt treatment. Furthermore, the suboptimal performance of immunological testing in patients referred for possible SLE has been highlighted. As a result, SLE remains largely a clinical diagnosis that is made after excluding alternative diagnoses. Diagnostic criteria can expedite diagnosis and treatment, but are not available for SLE. Thus, SLE classification criteria are often used, but strict adherence to these criteria could delay diagnosis. Therefore, while eagerly awaiting diagnostic criteria for this disease, we propose interim potential solutions to facilitate its diagnosis.

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Figure 1: Diagnostic steps in patients presenting with features suggestive of SLE.

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Acknowledgements

We wish to thank D. Vassilopoulos, I. Gergianaki and M. Drandaki for their thoughtful comments.

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All authors contributed to researching data for the article, discussing its content, writing the article and reviewing/editing it before publication.

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Correspondence to Dimitrios T. Boumpas.

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The authors declare no competing financial interests.

Supplementary information

Supplementary Table 1

The ACR and SLICC classification criteria for SLE. (DOCX 31 kb)

Supplementary Table 2

Diagnostic performance of individual features for established SLE patients seen in academic referral centers. (DOCX 27 kb)

Supplementary Table 3

Common cognitive biases and flaws in SLE diagnosis and management. (DOCX 24 kb)

Supplementary Figure 1

Fagan's normogram for calculating post-test probability after considering lupus laboratory tests. (DOCX 62 kb)

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Bertsias, G., Pamfil, C., Fanouriakis, A. et al. Diagnostic criteria for systemic lupus erythematosus: has the time come?. Nat Rev Rheumatol 9, 687–694 (2013). https://doi.org/10.1038/nrrheum.2013.103

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