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Application of the 2012 Systemic Lupus International Collaborating Clinics classification criteria to patients in a regional Swedish systemic lupus erythematosus register.

Ighe A, Dahlström Ö, Skogh T, Sjöwall C - Arthritis Res. Ther. (2015)

Bottom Line: SLICC-12 showed a diagnostic sensitivity of 94% (95% confidence interval (CI), 0.90 to 0.96) compared with 90% (95% CI, 0.85 to 0.93) for the updated set of ACR criteria from 1997 (ACR-97), whereas ACR-82 failed to identify every fifth true SLE case.However, the disease specificity of SLICC-12 reached only 74% (95% CI, 0.60 to 0.84) and did not change much when involvement of at least two different organs was required as an indicator of systemic disease.We confirm that SLICC-12 has advantages with regard to diagnostic sensitivity, whereas we found the diagnostic specificity to be surprisingly low.

View Article: PubMed Central - PubMed

Affiliation: Rheumatology/AIR, Department of Clinical and Experimental Medicine, Linköping University, University Hospital, SE-581 85, Linköping, Sweden. anna.ighe@gmail.com.

ABSTRACT

Introduction: In 2012, the Systemic Lupus International Collaborating Clinics (SLICC) network presented a new set of criteria (SLICC-12) to classify systemic lupus erythematosus (SLE). The present study is the first to evaluate the performance of SLICC-12 in an adult European study population. Thus, SLICC-12 criteria were applied to confirmed SLE cases in our regional SLE register as well as to individuals with a fair suspicion of systemic autoimmune disease who were referred to rheumatology specialists at our unit.

Methods: We included 243 confirmed SLE patients who met the 1982 American College of Rheumatology (ACR-82) classification criteria and/or the Fries 'diagnostic principle' (presence of antinuclear antibodies on at least one occasion plus involvement of at least two defined organ systems) and 55 controls with possible systemic autoimmune disease, including the presence of any SLE-related autoantibody.

Results: SLICC-12 showed a diagnostic sensitivity of 94% (95% confidence interval (CI), 0.90 to 0.96) compared with 90% (95% CI, 0.85 to 0.93) for the updated set of ACR criteria from 1997 (ACR-97), whereas ACR-82 failed to identify every fifth true SLE case. However, the disease specificity of SLICC-12 reached only 74% (95% CI, 0.60 to 0.84) and did not change much when involvement of at least two different organs was required as an indicator of systemic disease. In addition, SLICC-12 misclassified more of the controls compared to ACR-82, ACR-97 and Fries.

Conclusions: Establishing a standard definition of SLE continues to challenge lupus researchers and clinicians. We confirm that SLICC-12 has advantages with regard to diagnostic sensitivity, whereas we found the diagnostic specificity to be surprisingly low. To accomplish increased sensitivity and specificity figures, a combination of criteria sets for clinical SLE studies should be considered.

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Venn diagrams illustrating the distribution of confirmed systemic lupus erythematosus cases and controls for each separate classification ground. Venn diagrams demonstrate the distribution of the 243 confirmed (SLE) cases identified (A) by the 1982 American College of Rheumatology (ACR-82) criteria (blue), Fries (green) and SLICC-12 (pink), and (B) by ACR-82 criteria (blue), Fries and Holman diagnostic principle (Fries [7]) (green) and the 2012 Systemic Lupus International Collaborating Clinics criteria with SLICC-12 with a requirement for involvement of at least two organ systems for SLE diagnosis (SLICC-12:2) (red) (that is, with the requirement to involve at least two different organ systems). The 55 controls identified (C) by ACR-82 (blue), Fries (green) and SLICC-12 (pink) and (D) by ACR-82 (blue), Fries (green) and SLICC-12:2 (red). Controls who did not meet any of the classification grounds are indicated in black.
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Fig1: Venn diagrams illustrating the distribution of confirmed systemic lupus erythematosus cases and controls for each separate classification ground. Venn diagrams demonstrate the distribution of the 243 confirmed (SLE) cases identified (A) by the 1982 American College of Rheumatology (ACR-82) criteria (blue), Fries (green) and SLICC-12 (pink), and (B) by ACR-82 criteria (blue), Fries and Holman diagnostic principle (Fries [7]) (green) and the 2012 Systemic Lupus International Collaborating Clinics criteria with SLICC-12 with a requirement for involvement of at least two organ systems for SLE diagnosis (SLICC-12:2) (red) (that is, with the requirement to involve at least two different organ systems). The 55 controls identified (C) by ACR-82 (blue), Fries (green) and SLICC-12 (pink) and (D) by ACR-82 (blue), Fries (green) and SLICC-12:2 (red). Controls who did not meet any of the classification grounds are indicated in black.

Mentions: As demonstrated in Figure 1A, SLICC-12 identified 228 (94%) of 243 confirmed cases. To emphasize the systemic nature of SLE, we further increased the requirements for SLICC-12 by demanding involvement of at least two organ systems for SLE diagnosis (SLICC-12:2), but still with the exception of biopsy-proven LN (in combination with ANA/anti-dsDNA), which was considered a stand-alone item. As illustrated in Figure 1B, SLICC-12:2 identified 216 (89%) of 243 confirmed cases. Thus, 12 confirmed cases identified by SLICC-12 (5.2%) had involvement of only one organ system together with fulfilment of immunologic criteria.Figure 1


Application of the 2012 Systemic Lupus International Collaborating Clinics classification criteria to patients in a regional Swedish systemic lupus erythematosus register.

Ighe A, Dahlström Ö, Skogh T, Sjöwall C - Arthritis Res. Ther. (2015)

Venn diagrams illustrating the distribution of confirmed systemic lupus erythematosus cases and controls for each separate classification ground. Venn diagrams demonstrate the distribution of the 243 confirmed (SLE) cases identified (A) by the 1982 American College of Rheumatology (ACR-82) criteria (blue), Fries (green) and SLICC-12 (pink), and (B) by ACR-82 criteria (blue), Fries and Holman diagnostic principle (Fries [7]) (green) and the 2012 Systemic Lupus International Collaborating Clinics criteria with SLICC-12 with a requirement for involvement of at least two organ systems for SLE diagnosis (SLICC-12:2) (red) (that is, with the requirement to involve at least two different organ systems). The 55 controls identified (C) by ACR-82 (blue), Fries (green) and SLICC-12 (pink) and (D) by ACR-82 (blue), Fries (green) and SLICC-12:2 (red). Controls who did not meet any of the classification grounds are indicated in black.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4318183&req=5

Fig1: Venn diagrams illustrating the distribution of confirmed systemic lupus erythematosus cases and controls for each separate classification ground. Venn diagrams demonstrate the distribution of the 243 confirmed (SLE) cases identified (A) by the 1982 American College of Rheumatology (ACR-82) criteria (blue), Fries (green) and SLICC-12 (pink), and (B) by ACR-82 criteria (blue), Fries and Holman diagnostic principle (Fries [7]) (green) and the 2012 Systemic Lupus International Collaborating Clinics criteria with SLICC-12 with a requirement for involvement of at least two organ systems for SLE diagnosis (SLICC-12:2) (red) (that is, with the requirement to involve at least two different organ systems). The 55 controls identified (C) by ACR-82 (blue), Fries (green) and SLICC-12 (pink) and (D) by ACR-82 (blue), Fries (green) and SLICC-12:2 (red). Controls who did not meet any of the classification grounds are indicated in black.
Mentions: As demonstrated in Figure 1A, SLICC-12 identified 228 (94%) of 243 confirmed cases. To emphasize the systemic nature of SLE, we further increased the requirements for SLICC-12 by demanding involvement of at least two organ systems for SLE diagnosis (SLICC-12:2), but still with the exception of biopsy-proven LN (in combination with ANA/anti-dsDNA), which was considered a stand-alone item. As illustrated in Figure 1B, SLICC-12:2 identified 216 (89%) of 243 confirmed cases. Thus, 12 confirmed cases identified by SLICC-12 (5.2%) had involvement of only one organ system together with fulfilment of immunologic criteria.Figure 1

Bottom Line: SLICC-12 showed a diagnostic sensitivity of 94% (95% confidence interval (CI), 0.90 to 0.96) compared with 90% (95% CI, 0.85 to 0.93) for the updated set of ACR criteria from 1997 (ACR-97), whereas ACR-82 failed to identify every fifth true SLE case.However, the disease specificity of SLICC-12 reached only 74% (95% CI, 0.60 to 0.84) and did not change much when involvement of at least two different organs was required as an indicator of systemic disease.We confirm that SLICC-12 has advantages with regard to diagnostic sensitivity, whereas we found the diagnostic specificity to be surprisingly low.

View Article: PubMed Central - PubMed

Affiliation: Rheumatology/AIR, Department of Clinical and Experimental Medicine, Linköping University, University Hospital, SE-581 85, Linköping, Sweden. anna.ighe@gmail.com.

ABSTRACT

Introduction: In 2012, the Systemic Lupus International Collaborating Clinics (SLICC) network presented a new set of criteria (SLICC-12) to classify systemic lupus erythematosus (SLE). The present study is the first to evaluate the performance of SLICC-12 in an adult European study population. Thus, SLICC-12 criteria were applied to confirmed SLE cases in our regional SLE register as well as to individuals with a fair suspicion of systemic autoimmune disease who were referred to rheumatology specialists at our unit.

Methods: We included 243 confirmed SLE patients who met the 1982 American College of Rheumatology (ACR-82) classification criteria and/or the Fries 'diagnostic principle' (presence of antinuclear antibodies on at least one occasion plus involvement of at least two defined organ systems) and 55 controls with possible systemic autoimmune disease, including the presence of any SLE-related autoantibody.

Results: SLICC-12 showed a diagnostic sensitivity of 94% (95% confidence interval (CI), 0.90 to 0.96) compared with 90% (95% CI, 0.85 to 0.93) for the updated set of ACR criteria from 1997 (ACR-97), whereas ACR-82 failed to identify every fifth true SLE case. However, the disease specificity of SLICC-12 reached only 74% (95% CI, 0.60 to 0.84) and did not change much when involvement of at least two different organs was required as an indicator of systemic disease. In addition, SLICC-12 misclassified more of the controls compared to ACR-82, ACR-97 and Fries.

Conclusions: Establishing a standard definition of SLE continues to challenge lupus researchers and clinicians. We confirm that SLICC-12 has advantages with regard to diagnostic sensitivity, whereas we found the diagnostic specificity to be surprisingly low. To accomplish increased sensitivity and specificity figures, a combination of criteria sets for clinical SLE studies should be considered.

Show MeSH
Related in: MedlinePlus