Research Original Investigation
Progression of Cutaneous to Systemic Lupus
12. Isenberg DA, Rahman A, Allen E, et al. BILAG 2004: Development and initial validation of an updated version of the British Isles Lupus Assessment Group’s disease activity index for patients with systemic lupus erythematosus. Rheumatology (Oxford). 2005;44(7):902-906.
13. Yee CS, Farewell V, Isenberg DA, et al. British Isles Lupus Assessment Group 2004 index is valid for assessment of disease activity in systemic lupus erythematosus. Arthritis Rheum. 2007;56(12): 4113-4119.
14. Yee CS, Cresswell L, Farewell V, et al. Numerical scoring for the BILAG-2004 index. Rheumatology (Oxford). 2010;49(9):1665-1669.
Invited Commentary PRACTICE GAPS
Understanding How Cutaneous Lupus Erythematosus Progresses to Systemic Lupus Erythematosus Benjamin F. Chong, MD
Epidemiological studies have reported that up to 23% of patients with cutaneous lupus erythematosus (CLE) develop systemic lupus erythematosus (SLE), with some progressing over several years.1 As these data have been important in estimating one’s risk of systemic spread, how CLE progresses Related article page 291 to SLE in these patients is unknown. This article by Wieczorek et al2 seeks to address this practice gap by evaluating the severity of symptoms and SLE criteria that are seen and met in patients with CLE who develop SLE. Wieczorek et al2 prospectively follow 77 patients with CLE and determine that 17% eventually met criteria for SLE diagnosis. These 13 patients mostly fulfilled mucocutaneous criteria, and a minority of these (38%) displayed new moderate to severe systemic disease. These findings underscore that CLE progresses to SLE in a significant minority of patients and suggest that most patients with CLE who progress to SLE do not experience the severe manifestations of lupus, which have multiple clinical implications. First, it uncovers a practice gap because this insidious progression can be easily overlooked. Health care clinicians need to remain vigilant for concerning systemic symptoms and signs by conducting a complete review of systems (eg, joint swelling/pain in the small joints) at each clinic visit and ordering periodic laboratory tests such as complete blood cell counts with differentials and urinalyses at least once annually. A positive review of systems would prompt additional serologic tests (eg, antinuclear antibodies, anti–double-stranded DNA, antiSmith antibodies), but they are not recommended in the absence of systemic symptoms. Second, a new SLE diagnosis in CLE may ARTICLE INFORMATION Author Affiliation: Department of Dermatology, University of Texas Southwestern Medical Center, Dallas. Corresponding Author: Benjamin F. Chong, MD, Department of Dermatology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75309-9069 (ben.chong @utsouthwestern.edu).
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not require a specialist referral if they have skin-predominant disease. More than 30% of the patients with CLE that progressed to SLE in this study met criteria related to cutaneous findings with and without autoantibody abnormalities. Finally, we can educate these patients with this information to assuage concerns about their disease prognosis. Compared with an earlier epidemiological study,1 Wieczorek et al2 showed similar data showing that the mean duration between CLE and SLE diagnosis was 8.02 years. While this implies a gradual disease progression in these patients, this could also be due to delays in SLE diagnosis. Closing this practice gap would require improvements in educating health care clinicians on developing focused review of systems and evaluating laboratory test results. Widespread acceptance of this practice can occur with dermatologists embracing the responsibility of screening for SLE in patients with CLE. The article also identifies specific risk factors for progression such as positive antinuclear antibodies, widespread discoid lesions, female sex, and increased numbers of SLE criteria. While these particular characteristics will heighten the chances for systemic progression, the profile of these highrisk patients is not fully characterized. The Cutaneous Lupus Registry at University of Texas Southwestern Medical Center is prospectively studying patients with CLE who do and do not develop SLE to determine both clinical features and biomarkers that generate a thorough characterization of these highrisk patients. This form of “personalized” medicine can revolutionize treatment of patients with CLE because those at high risk for progression to SLE could be promptly started on antimalarial therapy, which can delay the onset of SLE.3
Published Online: January 29, 2014. doi:10.1001/jamadermatol.2013.9030. Conflict of Interest Disclosures: Dr Chong is an investigator for Daavlin Corporation. REFERENCES 1. Durosaro O, Davis MD, Reed KB, Rohlinger AL. Incidence of cutaneous lupus erythematosus, 1965-2005: a population-based study. Arch Dermatol. 2009;145(3):249-253.
2. Wieczorek IT, Propert KJ, Okawa J, Werth VP. Systemic symptoms in the progression of cutaneous to systemic lupus erythematosus [published online January 29, 2014]. JAMA Dermatol. doi:10.1001/jamadermatol.2013.9026. 3. James JA, Kim-Howard XR, Bruner BF, et al. Hydroxychloroquine sulfate treatment is associated with later onset of systemic lupus erythematosus. Lupus. 2007;16(6):401-409.
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Progression of Cutaneous to Systemic Lupus
12. Isenberg DA, Rahman A, Allen E, et al. BILAG 2004: Development and initial validation of an updated version of the British Isles Lupus Assessment Group’s disease activity index for patients with systemic lupus erythematosus. Rheumatology (Oxford). 2005;44(7):902-906.
13. Yee CS, Farewell V, Isenberg DA, et al. British Isles Lupus Assessment Group 2004 index is valid for assessment of disease activity in systemic lupus erythematosus. Arthritis Rheum. 2007;56(12): 4113-4119.
14. Yee CS, Cresswell L, Farewell V, et al. Numerical scoring for the BILAG-2004 index. Rheumatology (Oxford). 2010;49(9):1665-1669.
Invited Commentary PRACTICE GAPS
Understanding How Cutaneous Lupus Erythematosus Progresses to Systemic Lupus Erythematosus Benjamin F. Chong, MD
Epidemiological studies have reported that up to 23% of patients with cutaneous lupus erythematosus (CLE) develop systemic lupus erythematosus (SLE), with some progressing over several years.1 As these data have been important in estimating one’s risk of systemic spread, how CLE progresses Related article page 291 to SLE in these patients is unknown. This article by Wieczorek et al2 seeks to address this practice gap by evaluating the severity of symptoms and SLE criteria that are seen and met in patients with CLE who develop SLE. Wieczorek et al2 prospectively follow 77 patients with CLE and determine that 17% eventually met criteria for SLE diagnosis. These 13 patients mostly fulfilled mucocutaneous criteria, and a minority of these (38%) displayed new moderate to severe systemic disease. These findings underscore that CLE progresses to SLE in a significant minority of patients and suggest that most patients with CLE who progress to SLE do not experience the severe manifestations of lupus, which have multiple clinical implications. First, it uncovers a practice gap because this insidious progression can be easily overlooked. Health care clinicians need to remain vigilant for concerning systemic symptoms and signs by conducting a complete review of systems (eg, joint swelling/pain in the small joints) at each clinic visit and ordering periodic laboratory tests such as complete blood cell counts with differentials and urinalyses at least once annually. A positive review of systems would prompt additional serologic tests (eg, antinuclear antibodies, anti–double-stranded DNA, antiSmith antibodies), but they are not recommended in the absence of systemic symptoms. Second, a new SLE diagnosis in CLE may ARTICLE INFORMATION Author Affiliation: Department of Dermatology, University of Texas Southwestern Medical Center, Dallas. Corresponding Author: Benjamin F. Chong, MD, Department of Dermatology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75309-9069 (ben.chong @utsouthwestern.edu).
296
not require a specialist referral if they have skin-predominant disease. More than 30% of the patients with CLE that progressed to SLE in this study met criteria related to cutaneous findings with and without autoantibody abnormalities. Finally, we can educate these patients with this information to assuage concerns about their disease prognosis. Compared with an earlier epidemiological study,1 Wieczorek et al2 showed similar data showing that the mean duration between CLE and SLE diagnosis was 8.02 years. While this implies a gradual disease progression in these patients, this could also be due to delays in SLE diagnosis. Closing this practice gap would require improvements in educating health care clinicians on developing focused review of systems and evaluating laboratory test results. Widespread acceptance of this practice can occur with dermatologists embracing the responsibility of screening for SLE in patients with CLE. The article also identifies specific risk factors for progression such as positive antinuclear antibodies, widespread discoid lesions, female sex, and increased numbers of SLE criteria. While these particular characteristics will heighten the chances for systemic progression, the profile of these highrisk patients is not fully characterized. The Cutaneous Lupus Registry at University of Texas Southwestern Medical Center is prospectively studying patients with CLE who do and do not develop SLE to determine both clinical features and biomarkers that generate a thorough characterization of these highrisk patients. This form of “personalized” medicine can revolutionize treatment of patients with CLE because those at high risk for progression to SLE could be promptly started on antimalarial therapy, which can delay the onset of SLE.3
Published Online: January 29, 2014. doi:10.1001/jamadermatol.2013.9030. Conflict of Interest Disclosures: Dr Chong is an investigator for Daavlin Corporation. REFERENCES 1. Durosaro O, Davis MD, Reed KB, Rohlinger AL. Incidence of cutaneous lupus erythematosus, 1965-2005: a population-based study. Arch Dermatol. 2009;145(3):249-253.
2. Wieczorek IT, Propert KJ, Okawa J, Werth VP. Systemic symptoms in the progression of cutaneous to systemic lupus erythematosus [published online January 29, 2014]. JAMA Dermatol. doi:10.1001/jamadermatol.2013.9026. 3. James JA, Kim-Howard XR, Bruner BF, et al. Hydroxychloroquine sulfate treatment is associated with later onset of systemic lupus erythematosus. Lupus. 2007;16(6):401-409.
JAMA Dermatology March 2014 Volume 150, Number 3
Copyright 2014 American Medical Association. All rights reserved.
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