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

Albrecht, Joerg; Berlin, Jesse A.; Braverman, Irwin M.; Callen, Jeffrey P.; Costner, Melissa; Dutz, Jan P.; Fivenson, David P.; Franks, Andrew G.; Jorizzo, Joseph L.; Lee, L A; McCauliffe, Daniel P.; Sontheimer, Richard D.; Werth, Victoria P.

doi:10.1191/0961203304lu2020oa

Cited by 103 publications

(60 citation statements)

References 52 publications

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“…On the other hand, photosensitivity, i.e. "unusual reaction to sunlight by a patient's history or by physician observation", was considered as a separate diagnostic criterion of SLE [14,15], which might lead to a faulty increase in the number of positive SLE criteria in selected patients, as "malar rash" is often indistinguishable from photosensitivity, and therefore these criteria were not independent [23]. These shortcomings have been substantially improved in the new diagnostic criteria proposed by Petri et al [24], as they recognized many previously excluded cutaneous LE manifestations, including SCLE and LE tumidus.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics of cutaneous lupus erythematosus

Szczęch

Rutka

Samotij

et al. 2016

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13 IntroductionLupus erythematosus (LE) is a chronic autoimmune disease with a wide spectrum of presenting symptoms ranging from mild cutaneous manifestations in localized cutaneous LE (CLE) to severe, life-threatening internal organ damage in systemic LE (SLE). The etiology of LE remains unknown, although the environmental, genetic, viral and hormonal factors are taken into consideration as probable causes or precipitating factors of this disease [1,2].Skin involvement is seen in about 70-85% of all LE patients [3]. Sometimes skin involvement is the only manifestation of LE, while in other cases it could rather be a mild bystander of severe internal involvement. Isolated CLE is a rare disease, albeit it is still about 2 to 3 times more frequent than SLE [4]. Cutaneous features of LE can be classified into LE-specific and LE-nonspecific ones. LEspecific skin lesions usually appear only in patients with LE and thus can be handled as diagnostic ones (e.g. "ma- Aim: To analyze the clinical presentation of cutaneous variants of lupus erythematosus in terms of skin lesion spectrum and extracutaneous involvement. Material and methods: A total of 64 patients with cutaneous LE (CLE) were included. The study was based on the "Core Set Questionnaire" developed by the European Society of Cutaneous Lupus Erythematosus (EUSCLE). Clinical severity of skin lesions was evaluated with the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). All results were subjected to statistical analysis. Results: Fifteen (23.4%) patients had an acute CLE (ACLE), 26 (40.6%) subacute CLE (SCLE) and 21 (32.8%) chronic CLE (CCLE). Two (3.2%) individuals only demonstrated urticarial vasculitis as a cutaneous manifestation of LE and these patients were excluded. Patients with ACLE were characterized by the earliest onset of the disease (mean age of 31.9 ±15.0 years; p < 0.001). On average, 4.8 ±1.8 criteria of systemic LE were found in the ACLE group compared to 2.7 ±1.3 criteria in SCLE and 2.5 ±1.5 criteria in CCLE (p < 0.001). The highest activity of skin lesions according to CLASI was found in the SCLE group (p = 0.002). On the other hand, the most severe skin damage was observed in CCLE (p < 0.01). Conclusions: Each variant of CLE differs significantly from the others in respect of various aspects of clinical manifestations. Due to a number of different variants of LE skin lesions, a unified classification of CLE still remains a challenge.

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Section: Discussionmentioning

confidence: 99%

Clinical characteristics of cutaneous lupus erythematosus

Szczęch

Rutka

Samotij

et al. 2016

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“…12 While specificity was usually considered satisfactory, the sensitivity of the ACR criteria has been seen as suboptimal, in new-onset and childhood SLE in particular. 13 Moreover, the 1997 revision was never formally tested.…”

Section: Sle Classification-the Acr and The Slicc Criteriamentioning

confidence: 99%

Toward new criteria for systemic lupus erythematosus—a standpoint

Aringer

Dörner

Leuchten

et al. 2016

Lupus

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While clearly different in their aims and means, classification and diagnosis both try to accurately label the disease patients are suffering from. For systemic lupus erythematosus (SLE), this is complicated by the multi-organ nature of the disease and by our incomplete understanding of its pathophysiology. Hallmarks of SLE are the presence of antinuclear antibodies (ANA), and multiple immune-mediated organ symptoms that are largely independent. In an attempt to overcome limitations of the current sets of SLE classification criteria, a new fourphase approach is being developed, which is jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). This review attempts to delineate the performance of the current sets of criteria, the reasons for the decision for classification, and not diagnostic, criteria, and to provide a background of the current approach taken. Lupus (2016) 25, 805-811.

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“…Concluding that the use of photosensitivity as a classification criterion for SLE remains questionable, at least when it is assessed by patient or physician history according to the ACR criteria. Moreover, the "malar rash," a further ACR criterion used for the classification of SLE, is often indistinguishable from photosensitivity and, therefore, the two criteria are not completely independent [98].…”

Section: Discussionmentioning

confidence: 99%

Cutaneous manifestations of systemic lupus erythematosus

2018

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Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. The skin is one of the target organs most variably affected by the disease. The American College of Rheumatology (ACR) established 11 criteria as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. Cutaneous lesions account for four of these 11 revised criteria of SLE. Skin lesions in patients with lupus may be specific or nonspecific. This paper covers the SLE-specific cutaneous changes: malar rash, discoid rash, photosensitivity, and oral mucosal lesions as well as SLE nonspecific skin manifestations, their pathophysiology, and management. A deeper thorough understanding of the cutaneous manifestations of SLE is essential for diagnosis, prognosis, and efficient management. Thus, dermatologists should cooperate with other specialties to provide optimal care of SLE patient.

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

Cited by 103 publications

References 52 publications

Clinical characteristics of cutaneous lupus erythematosus

Clinical characteristics of cutaneous lupus erythematosus

Toward new criteria for systemic lupus erythematosus—a standpoint

Cutaneous manifestations of systemic lupus erythematosus

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