Background/Purpose: Systemic lupus erythematosus (SLE) is a prototype autoimmune disease. The most widely used classification criteria for SLE were those developed by the American College of Rheumatology (ACR) in 1982 and revised by a committee in 1997, but not validated in that revision. The Systemic Lupus Collaborating Clinics (SLICC) revised the ACR SLE classification criteria and validated new criteria in order to improve clinical relevance and incorporate new knowledge in SLE immunology .

 To assess sensitivity and specificity of revised and validated new SLICC SLE classification criteria in a cohort of Juvenile SLE patients.

Methods: The SLICC criteria rule for SLE classification requires: 1) four criteria, with at least one clinical criterion and one immunologic criterion or 2) lupus nephritis alone in the presence of ANA or anti-DNA antibodies. Seventeen criteria were identified. Cases were JSLE patients who were attending a single tertiary center in the past 10 years. JSLE had been diagnosed on clinical and immunological grounds by experienced pediatric rheumatologist. Controls were patients with rheumatic diseases other than SLE: Juvenile Idiopathic Arthritis (JIA); Juvenile Dermatomyositis (JDM), Autoimmune Hepatitis (AH) and Juvenile Systemic Sclerosis (JSS). Criteria were reviewed from prospectively developed databases and medical records by pediatric rheumatologists in order to establish the number and frequency of new criteria fulfilled by each patient. Descriptive statistics were used to characterize both patients groups. Summary statistics included overall sensitivity and specificity. McNemar`s test was used to assess differences between ACR 1997 criteria and SLICC criteria with respect to accuracy.

Results: Cases: 107 patients with JSLE were included (F: 89 M: 18 ), age at onset: 12 (3-16) yo. Controls: 102 patients with JIA  (36 patients, systemic 20, polyarticular 16); JDM (28), AH (28) and JSS (10), F:76  M: 26 , age at onset: 11 (2-16) yo. SLICC SLE criteria sensitivity was 100 % vs 86% ACR 1997 criteria, while specificity was 98% vs 96% (p=0.009). Six patients with a clinical diagnosis of JSLE were correctly classified by SLICC but not by ACR criteria.

Table lists the sensitivity and specificity of each criterion in SLICC SLE criteria

 

SLICC SLE criteria	Sensitivity %	Specificity %
Acute cutaneous lupus	61	100
Chronic cutaneous lupus	2	100
Oral ulcers	11	100
Nonscarring alopecia	14	98
Synovitis	65	68
Serositis	13	98
Renal	56	99
Neurologic	11	100
Hemolytic anemia	21	98
Leukopenia or Lymphopenia	34	95
Thrombocytopenia	31	92
ANA	100	60
Anti-DNA	63	100
Anti-Sm	30	100
Antiphospholipid antibodies	33	100
Low complement	91	90
Direct Coombs test	29	98

Conclusion: The SLICC new criteria performed better than the ACR 1997 criteria in a cohort of patients with JSLE. These new criteria allowed better accuracy than previous criteria in some variables such as low complement, anti-DNA,  acute cutaneous lupus, ANA, and renal involvement.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/accuracy-of-systemic-lupus-international-collaborating-clinics-classiffication-criteria-applied-to-juvenile-systemic-lupus-erythematosus-patients/