Review of Classification Criteria for Systemic Lupus Erythematosus

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Goals of classification criteria

In SLE, the population studied affects the results obtained. The prevalence of various organ manifestations and autoantibodies differ by ethnicity. African Americans who have SLE are more likely than whites to have discoid lupus, lupus nephritis, anti-Sm, and anti-RNP. Whites who have SLE are more likely to have photosensitivity, malar rash, oral ulcers, and antiphospholipid antibodies [4]. Recent data suggest that SLE differs among nations, even beyond ethnicities. Central nervous system SLE,

Ideal classification criteria

Five rules to be met in constructing classification criteria for SLE were summarized by Fries [12]:

  • 1.

    The gold standard for diagnosis (or classification) is established by highly experienced clinicians and is confirmed by: stability of the diagnosis over time and by independent review by committee members.

  • 2.

    Consecutively treated patients and multiple institutions are used to minimize selection bias.

  • 3.

    Control populations are chosen to represent the diagnostic problem area that will be used in selecting

American College of Rheumatology 1982 criteria

The first classification criteria for SLE was established in 1971 and required 4 of 14 criteria [13]. In 1982, the criteria were revised to include advances in serologic testing (ANA and anti-dsDNA) and to take advantage of improved biostatistical techniques [6]. These criteria are shown in Table 1. Two items, alopecia and Raynaud's phenomenon, were removed from the 1971 criteria. The 1982 criteria have been validated [14], [15], [16], [17].

The interpretation of some of the definitions is

Weighted criteria

It seems intuitively obvious that some criteria are more important than others (eg, lupus nephritis), and therefore, should be accorded more importance. In 1984, Clough et al applied Bayes' theorem to develop such a weighting system [19]. They assumed that various criteria occurred in individual patients independently of each other, although this is unproven. A positive (or negative) score for the presence/absence of each criterion was calculated and summed. A probability curve was constructed

Classification trees

Classification criteria can be presented in traditional and classification tree (recursive partitioning) formats. Using the same data set as the 1982 criteria, but with some standardization of laboratory values for ANA and complement, Edworthy et al derived several sets of classification trees [21]. The simplest tree (Fig. 2) demonstrated that just two criteria, immunologic criterion and malar rash, performed as well as the 1982 criteria set. The second classification tree (Fig. 3) contained

Cutaneous criteria

Although all other organ systems receive only one criterion, there are four dermatologic criteria for SLE: malar rash, discoid rash, photosensitivity, and oral ulcers. Malar rash and photosensitivity correlate highly. They may be difficult to interpret, even if witnessed by a physician. Several other conditions, including acne rosacea, seborrheic dermatitis, actinic damage, and steroid erythema can be mistaken for a malar rash. Polymorphous light eruption, allergic reactions, and solar

Revision of the American College of Rheumatology classification criteria: role of Systemic Lupus International Collaborating Clinics

Because of the limitations of ACR classification criteria, the need to include new laboratory assays, and the need to address the omission of hypocomplementemia, the Systemic Lupus International Collaborating Clinics (SLICC) has undertaken the revision of the criteria. This process first involved defining all the potential candidate criteria by consensus. The frequency of these potential criteria then was determined in existing cohorts [5]. These potential criteria now will be tested in

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