ARA and EADV criteria for classification of systemic lupus erythematosus in patients with cutaneous lupus erythematosus

Objective: To verify (1) how many patients with cutaneous lupus erythematosus (CLE) fulfill 4 or more American Rheumatism Association (ARA) and European Academy of Dermatology and Venereology (EADV) criteria for classifi cation of systemic lupus erythematosus (SLE); (2) which criteria are mostly fulfilled; (3) the severity of the disease in patients fulfilling criteria; (4) how many patients with systemic involvement fail to fulfill 4 ARA and EADV criteria. Methods: We studied 207 patients with chronic and subacute CLE, classified according to ARA and EADV criteria. Results: Twenty-four patients with localized discoid (L-DLE; 21.8%), 22 with disseminated discoid (D-DLE; 30.5%) and 7 with subacute CLE (SCLE; 28%) had 4 or more ARA criteria. With EADV criteria, these figures fell to 7 (6.4%), 7 (9.7%) and 6 (24%), respectively. Only 3 L-DLE (2.7%), 5 D-DLE (6.9%) and 3 SCLE cases (12%) defined as SLE by ARA criteria and 1, 3 and 3, respectively, by EADV criteria had a renal or neurological disorder, hemolytic anemia and/or thrombocytopenia, vasculitis or serositis. ARA criteria did not classify 7 patients with a similar visceral involvement, while EADV criteria failed in 11 patients. Conclusion: In our patients, ARA criteria showed a sensitivity of 88%, a specificity of 79%, a positive predictive value of 56% and a negative predictive value of 96%. EADV criteria showed a sensitivity of only 64%, but a specificity of 93%, a positive predictive Value of 61% and a negative predictive value of 94%. ARA criteria should not be used in CLE patients as they are too sensitive, poorly specific and altogether misleading. EADV criteria are more specific, but less sensitive.