The Influence of the 1997 Updated Classification Criteria for Systemic Lupus Erythematosus: Epidemiology, Disease Presentation, and Patient Management

Abstract

ABSTRACT. Objective. The 1997 update of the American College of Rheumatology classification criteria (ACR97) for systemic lupus erythematosus (SLE) has not been validated. We determined to what extent their introduction influenced the epidemiology and clinical characteristics of the disease in northern Norway. Methods. Annual incidence and point-prevalence rates, clinical manifestations, and outcome were determined in an inception cohort of patients with SLE in northern Norway, included between 1996 and 2006, using ACR97 criteria (97acr). These findings were compared with a cohort from the same area enrolled 1978-1995 using the 1982 revised criteria ACR82 (82acr). Results. The mean annual incidence of SLE was 3.00 for cohort 97acr (n = 58) versus 2.63 for cohort 82acr (n = 81) (p = 0.5). All patients in the 97acr cohort also fulfilled the 1982 criteria; however, significantly fewer patients presented with discoid rash [odds ratio (OR) 0.31)], arthritis (OR 0.24), renal (OR 0.28) or hematological disorder (OR 0.27), and significantly more with anti-dsDNA (OR 2.57) and antiphospholipid antibodies (OR 27.9). Initial treatment with intravenous pulse methylprednisolone (OR 9.23), azathioprine (OR 6.32), and low-dose aspirin (OR 20.9) was increased in cohort 97acr. Five-(95.2%) and 10-year survival (91.9%) rates were also improved for cohort 97acr. Conclusion. The ACR97 criteria set has construct validity compared to the ACR82 criteria set. SLE incidence remains unchanged in northern Norway, but a significant reduction of renal disease and further improvements in survival rates occurred simultaneously with increased serological surveillance with ELISA-based assays and early immunosuppressive and anticoagulant therapy.

Antimalarials in the treatment of systemic lupus erythematosus: a registry-based cohort study in Denmark

“…Previous studies have found a prevalence of lupus nephritis between 20% and 30%. 10,41 In recent years, however, a decrease has been observed, 42 and this finding is in agreement with the findings in our study. Misclassification of cases might explain the relatively few cases of lupus nephritis in our study.…”

Disease activity and damage accrual during the early disease course in a multinational inception cohort of patients with systemic lupus erythematosus

“…Antiphospholipid Ab have become part of the classification process for SLE, 13 and the overall prevalence of aPL (48%) agrees with other series. 30,31 While inclusion of aPL as a classification criterion has not led to large changes in the clinical spectrum of SLE at diagnosis, 12 they are likely connected to the remarkably high (7.5%) overall frequency of ischaemic events in this cohort with a mean age of 35 years. The frequencies for vascular events in the first years of disease (cerebrovascular events 4%, venous thrombosis 2% and angina pectoris 1%) confirm the need for better markers to identify patients at vascular risk, as current aPL assays are not specific enough.…”

“…6,[40][41][42] This will likely require use of immunosuppressive therapy for some time to come, even though the potential benefits of such treatment are offset by increased infectious complications. 8 Thus, in addition to increased efforts for early diagnosis at a less severe disease stage, 12 there is a clear need for less toxic but efficient ways of reducing early disease activity, such as through B-cell depletion or inactivation.…”

“…[4][5][6][7][8] The overall disease activity course remains unpredictable with flares continuing to occur well into the second decade of disease. to genetic and/or geographic influences, 10 that changes in SLE phenotype can occur within a single region 11,12 and that patient management options are steadily expanding, 13 there is a continued need for surveillance of the clinical characteristics and complications in SLE. This study describes the presentation and early disease course in a contemporary multinational European inception cohort and investigates current presentation and disease severity with regard to possible regional differences and the risk of organ damage and early mortality.…”

Thrombopoietin levels in systemic lupus erythematosus are linked to inflammatory cytokines, but unrelated to thrombocytopenia or thrombosis

“…[28][29][30] For the current study, clinical and routine laboratory data as well as blood samples for storage were collected simultaneously during an extended outpatient visit. Thrombocytopenia was defined as platelet counts <150 and was considered mild with platelet counts between 100-150, moderate between 100-50 and severe <50.…”