Avoiding misclassification of thrombotic primary antiphospholipid syndrome as systemic lupus erythematosus (SLE): What are the best-performing SLE classification criteria?

Author(s)

Signorelli, Flavio ^{[1, 2]} ; Balbi, Gustavo Guimaraes Moreira ^{[1, 3]} ; Bonfa, Eloisa ^[1] ; Borba, Eduardo F. ^[1] ; Andrade, Danieli Castro de Oliveira ^[1]

Affiliation

^[1] Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Rheumatol Div, Av Dr Arnaldo 455, Third Floor, LIM 17, BR-01246903 Sao Paulo, SP - Brazil

^[2] Univ Estado Rio De Janeiro, Hosp Univ Pedro Ernesto, Rheumatol Div, Rio De Janeiro - Brazil

^[3] Univ Fed Juiz De Fora, Univ Hosp, Rheumatol Div, Juiz De Fora - Brazil

• Document type: Journal article
• Source: Lupus; v. 30, n. 11 JUL 2021.
• Web of Science Citations: 0

Abstract

Background Systemic lupus erythematosus (SLE) and Primary Antiphospholipid Syndrome (PAPS) overlap clinical and immunological features. Therefore, misclassification of PAPS patients as SLE is a concern. The ACR/EULAR 2019 SLE classification has never been studied in PAPS. Objective To verify if the ACR/EULAR 2019 SLE classification can correctly classify a PAPS patient as not having SLE and compare its performance with the SLICC 2012 SLE classification. Methods: One-hundred thrombotic PAPS patients who fulfilled the Sidney criteria were consecutively screened and those who attended the inclusion criteria were submitted to ACR/EULAR 2019 and SLICC 2012 classifications. Results Sixty-seven PAPS patients were included in this study. The majority was female (89.6%) with median age at study inclusion of 45 years (35-53) and median PAPS disease duration of 13 years (8-19). PAPS correct classification was observed more often with ACR/EULAR 2019 than SLICC 2021 criteria (94.0% vs. 64.2%; p < 0.001). The 4 misclassified patients in ACR/EULAR 2019 were also misclassified in SLICC 2012. The comparison of misclassified patients to those correctly not classified as SLE resulted, for both criteria, in higher frequencies of hematological domain {[}ACR/EULAR 2019 (100% vs. 28.6%, p = 0.010) and SLICC 2012 (95.8% vs. 11.6%, p < 0.001)]. Further analysis of hematological manifestations revealed that for the ACR/EULAR 2019 leukopenia (100% vs. 22.2%, p = 0.004) and for the SLICC 2012 leukopenia/lymphopenia (91.7% vs. 7%, p < 0.001) were more frequent in misclassified group. Proteinuria (20.8% vs. 0%, p = 0.004) and low complement (45.8% vs. 20.9%, p = 0.033) were also more often observed in the incorrectly SLICC 2012 classified patients. Conclusion ACR/EULAR 2019 had high accuracy for distinguishing PAPS from SLE, whereas the SLICC 2012 incorrectly classified more than one third of the PAPS patients as having SLE. (AU)

• FAPESP's process: 15/03756-4 - Assessment of relevance of blood levels of drugs in the monitoring rheumatic autoimmune diseases: safety, effectiveness and adherence to therapy
• Grantee: Eloisa Silva Dutra de Oliveira Bonfá
• Support Opportunities: Research Projects - Thematic Grants