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Abstract Number: 0521

Avoiding Misclassification of Primary Antiphospholipid Syndrome as Systemic Lupus Erythematosus: What Are the Best-performing SLE Classification Criteria?

Flavio Signorelli1, Gustavo Balbi2, Eloisa Bonfa3, Eduardo Borba4 and Danieli Andrade5, 1Universidade de São Paulo, Rio de janeiro, Rio de Janeiro, Brazil, 2Universidade de São Paulo, Juiz de Fora, Minas Gerais, Brazil, 3Hospital das Clínicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Sao Paulo, Brazil, 4Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil, 5University of Sao Paulo, Sao Paulo, Brazil

Meeting: ACR Convergence 2020

Keywords: antiphospholipid syndrome, classification criteria, Systemic lupus erythematosus (SLE)

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Session Information

Date: Saturday, November 7, 2020

Session Title: Antiphospholipid Syndrome Poster

Session Type: Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Primary Antiphospholipid Syndrome (PAPS) patients, when submitted to Systemic Lupus Erythematosus (SLE) classification criteria, can be misclassified. The new 2019 ACR/EULAR classification criteria have never been applied for PAPS patients. The main objective of this study is to evaluate the risk of misclassifying thrombotic PAPS (Sidney) as SLE in a cohort of a tertiary Hospital.

Methods: A retrospective analysis of our PAPS cohort was performed using electronic chart database. The three current classification criteria for SLE (ACR 1997, SLICC 2012 and 2019 ACR/EULAR) were applied in parallel focusing on the latter. Flowchart of the study is shown in Figure 1. Categorical variables were analyzed by x2 or two-tailed Fisher’s exact test, and continuous variables were analyzed by Student’s T test or Mann–Whitney-U test, as applicable. The misclassification rates of the three different criteria were compared using McNemar’s test. A p-value of < 0.05 was considered statistically significant.

Results: All patients had thrombotic PAPS and 55.6% presented obstetric criteria manifestations. Venous and arterial thrombosis were detected in 69.4% and 39.8%, respectively. Ninety-eight PAPS patients were submitted to ACR 1997 and 2019 ACR/EULAR. SLICC 2012 was applied in only 83 patients due to missing data (lack of direct Coombs test). Our patients were mostly female (87.8%), with a median age at study inclusion of 46 years (38-56) and median disease duration of 14 years (10-21). LA was the most frequent antiphospholipid antibody, identified in 91 patients (93.8%). Main characteristics of the studied patients are shown in Table 1. The comparison of different criteria two-by-two according to their rates of misclassification found that 2019 ACR/EULAR and ACR 1997 performed similarly (N=98; 6.1 vs. 6.1%, p=1.00). On the other hand, SLICC 2012 demonstrated a poor performance compared to both 2019 ACR/EULAR (N=83, 36.1 vs. 6.1%, p< 0.001) and ACR 1997 criteria (N=83, 36.1 vs. 6.1%, p< 0.001). When the 3 classification criteria were used simultaneously, only 1 patient was misclassified as SLE. Hematological manifestations were the most frequent criteria related to misclassification in PAPS patients, encompassing 100% of misclassified patients (vs. 52.2%, p=0.025) in ACR 1997, 90% (vs. 11.3%, p< 0.001) in SLICC 2012 and 71.4% (vs. 18.6%, p=0.01) in 2019 ACR/EULAR.

Conclusion: 2019 ACR/EULAR is very accurate in differentiating PAPS from SLE, with only 6.1% of misclassification rates (similar to those observed in ACR 1997 and lower than those found in SLICC 2012).


Disclosure: F. Signorelli, None; G. Balbi, None; E. Bonfa, Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP #2015/03756-4), 2, Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq #305068/2014-8), 2; E. Borba, None; D. Andrade, None.

To cite this abstract in AMA style:

Signorelli F, Balbi G, Bonfa E, Borba E, Andrade D. Avoiding Misclassification of Primary Antiphospholipid Syndrome as Systemic Lupus Erythematosus: What Are the Best-performing SLE Classification Criteria? [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/avoiding-misclassification-of-primary-antiphospholipid-syndrome-as-systemic-lupus-erythematosus-what-are-the-best-performing-sle-classification-criteria/. Accessed March 7, 2021.
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