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Dermatology position paper on the revision of the 1982 ACR criteria for systemic lupus erythematosus

Department of Dermatology, University of Pennsylvania, PA, USA

J A Berlin

Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, PA, USA

I M Braverman

Department of Dermatology, Yale Medical School, New Haven, CT, USA

J P Callen

Division of Dermatology, Department of Medicine, University of Louisville, Lousiville, KY, USA

M I Costner

Department of Dermatology, Southwestern Medical School, Dallas, TX, USA

J Dutz

Division of Dermatology, University of British Columbia, Vancouver, Canada

D Fivenson

Private Practice, Ann Arbor, MI, USA

A G Franks

Department of Dermatology, NYU Medical Center, New York, NY, USA

J L Jorizzo

Department of Dermatology, Bowman Gray School of Medicine, Winston-Salem, NC, USA

L A Lee

Dermatology Service, Department of Medicine, Denver Health Medical Center, Denver, CO, USA, Departments of Dermatology and Medicine, University of Colorado School of Medicine, Denver, CO, USA

D P McCauliffe

Private Practice, Rutland, VT, USA

R D Sontheimer

Department of Dermatology, University of Iowa, Iowa City, IO, USA

V P Werth

Department of Dermatology, University of Pennsylvania, PA, USA, Dermatology Service, Veterans Hospital Philadelphia, Philadelphia, PA, USA, werth{at}mail.med.upern.edu

The 1982 ACR classification criteria have become de facto diagnostic criteria for systemic lupus erythematosus (SLE), but a review of the criteria is necessary to include recent diagnostic tests. The criteria were not developed with the help of dermatologists, and assign too much weight to the skin as one expression of a multiorgan disease. Consequently, patients with skin diseases are classified as SLE based mostly on skin symptoms. We discuss specific problems with each dermatologic criterion, but changes must await a new study. We suggest the following guidelines for such a study, aimed at revision of the criteria. 1) The SLE patient group should be recruited in part by dermatologists. 2) The study should evaluate an appropriate international ethnic/racial mix, including late onset SLE as well as pediatric patients. 3) All patients should have current laboratory and clinical evaluations, as suggested in the paper, to assure the criteria can be up-to-date. This includes anti-SS-A and anti-SS-B antibodies and skin biopsies for suspected cutaneous lupus erythematosus except for nonscarring alopecia and oral ulcers. 4) The study should be based on a series of transparent power calculations. 5) The control groups should represent relevant differential diagnoses in numbers large enough to assess diagnostic problems that might be specific to these differential diagnoses. In order to demonstrate specificity of the criteria with a 95% confidence interval between 90 and 100%, each control group of the above should have at least 73 patients.

Key Words: classification • cutaneous lupus erythematosus • diagnosis • diagnostic criteria • discoid lupus erythematosus • study design • subacute cutaneous lupus erythematosus • systemic lupus erythematosus

Lupus, Vol. 13, No. 11, 839-849 (2004)
DOI: 10.1191/0961203304lu2020oa


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