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Original Investigation |

Systemic Symptoms in the Progression of Cutaneous to Systemic Lupus Erythematosus

Isabela T. Wieczorek, MD1,2; Kathleen J. Propert, ScD3; Joyce Okawa, RN1,2; Victoria P. Werth, MD1,2
[+] Author Affiliations
1Department of Dermatology, Philadelphia Veterans Affairs Medical Center, Philadelphia, Pennsylvania
2Department of Dermatology, University of Pennsylvania, Philadelphia
3Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania, Philadelphia
JAMA Dermatol. 2014;150(3):291-296. doi:10.1001/jamadermatol.2013.9026.
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Importance  Patients with cutaneous lupus erythematosus (CLE) who develop systemic lupus erythematosus (SLE) may have few and mild systemic symptoms.

Objective  To characterize the types and severity of systemic symptoms in a longitudinal cohort of patients with CLE.

Design, Setting, and Participants  Prospective, longitudinal cohort study of 77 patients with CLE who presented between January 2007 and April 2011 at a university autoimmune skin disease clinic.

Main Outcomes and Measures  Systemic symptoms and severity were determined from data recorded at each study visit and from medical records.

Results  Of 77 patients with CLE, 13 (17%) went on to meet criteria for SLE, with a mean (SD) time from CLE diagnosis to SLE of 8.03 (6.20) years. Of the 13 patients, 1 (8%) solely met the mucocutaneous American College of Rheumatology (ACR) criteria of malar rash, discoid rash, photosensitivity, and oral ulcers, and 3 (23%) met the mucocutaneous ACR criteria plus positive antinuclear and other antibody titers. After a mean (SD) follow-up time of 2.81 (1.34) years, only 5 of the 13 patients with CLE (38%) who progressed to meet SLE criteria developed moderate to severe additional systemic disease.

Conclusions and Relevance  Patients with CLE who developed SLE during our study did so mostly by meeting the mucocutaneous ACR criteria, and the majority developed none to mild additional systemic disease during the study period. Thus, our study suggests that a small percentage of patients with CLE eventually develop SLE and that even if they do, most patients will have mild systemic disease.

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