Prognostic implications of active discoid lupus erythematosus and malar rash at the time of diagnosis of systemic lupus erythematosus: Results from a prospective cohort study

Drucker, Aaron M.; Su, Jiandong; Mussani, Farheen; Siddha, Sanjay; Gladman, Dafna D.; Urowitz, Murray B.

doi:10.1177/0961203315610645

Cited by 13 publications

(14 citation statements)

References 16 publications

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“…Lupus-specific cutaneous manifestations are important in relation to the development of systemic involvement and ultimate prognosis. [1][2][3] Among juvenile patients with SLE, those with acute cutaneous lupus erythematosus or non-scarring alopecia were more likely to develop arthralgia, while mucosal ulcers were associated with a higher risk of leucopenia. 1 In adult patients with SLE, the presence of malar rash was indicative of more severe systemic disease, while discoid lupus appeared to be associated with a decreased incidence of renal disease 2 3 but an increased Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI).…”

Section: I-introductionmentioning

confidence: 99%

Cutaneous vasculitis in SLE

Goldman

Petri

2020

Lupus Sci Med

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ObjectivesWe determined the temporal association between clinical and serological disease manifestations and development of cutaneous small vessel vasculitis in a large prospective multiethnic cohort.MethodsPatients with SLE diagnosed according to the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria or the revised classification criteria as defined by the American College of Rheumatology (ACR) were enrolled in the Hopkins Lupus Cohort. Cutaneous small vessel vasculitis was determined as a component of the Systemic Lupus Erythematosus Disease Activity Index. SLE-associated cutaneous small vessel vasculitis lesions were reported clinically. They presented as punctate lesions, palpable purpura, tender erythematous plaques or macules with or without necrosis. No histopathological diagnosis was pursued to confirm the diagnosis of vasculitis or to differentiate it from other causes of digital lesions in patients with SLE. Disease manifestations that preceded the first occurrence of cutaneous small vessel vasculitis lesions were analysed using Kaplan-Meier. Cox regression analysis was used to assess the relationship between baseline clinical and immunological manifestations and the development of cutaneous small vessel vasculitis. We adjusted for gender, race and age at SLE diagnosis.ResultsA total of 2580 patients were studied: 52.4% were Caucasian and 39.4% were African-American. The mean age of the cohort was 45.5±14.5 years. The mean years of cohort follow-up was 7.9±7.6. Cutaneous small vessel vasculitis was observed in 449 (17.3%). The mean time to cutaneous vasculitis after SLE diagnosis was 4.78 years (95% CI 3.96 to 5.60). At least 159 (35%) patients had recurrences of cutaneous vasculitis lesions. Discoid rash, Raynaud’s phenomenon, myositis, anaemia, Coombs’ positivity, leucopenia, anti-Smith and anti-RNP (Ribonucleoprotein) were significantly associated with the development of cutaneous vasculitis. The SLICC/ACR Damage Index score was higher in patients with cutaneous vasculitis compared with those without cutaneous vasculitis.ConclusionsCutaneous vasculitis is frequent (17.3%) and often recurrent (35%). African-Americans are at higher risk of developing cutaneous small vessel vasculitis than Caucasians. Clinical presentations such as myositis and haematological manifestations are predictors of cutaneous vasculitis development. The presence of cutaneous vasculitis is associated with increased organ damage.

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Section: I-introductionmentioning

confidence: 99%

Cutaneous vasculitis in SLE

Goldman

Petri

2020

Lupus Sci Med

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“…The presence of malar rash was indicative of more severe disease in one study. 40 From multivariate analyses, we could identify novel significant associations between presenting cutaneous manifestations and an increased risk of development of systemic involvement in both groups. Among jSLE patients, ACLE, mucosal ulcer, non-scarring alopecia, and cutaneous vasculitis were independent risk factors for developing certain dissimilar systemic manifestations.…”

Section: Discussionmentioning

confidence: 98%

Skin signs in juvenile- and adult-onset systemic lupus erythematosus: clues to different systemic involvement

Chottawornsak

Rodsaward

Suwannachote

et al. 2018

Lupus

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Objective We aim to explore the differences of skin signs between juvenile- and adult-onset systemic lupus erythematosus and to identify their associations to the development of systemic involvement. Methods A retrospective chart review of 377 systemic lupus erythematosus patients was performed. Results In total, 171 patients with juvenile systemic lupus erythematosus and 206 with adult systemic lupus erythematosus were studied. All patients were of Southeast Asian descent. The mean duration of follow up was 8.18 ± 6.19 and 9.36 ± 7.68 years for juvenile systemic lupus erythematosus and adult systemic lupus erythematosus, respectively. At diagnosis, most patients presented with acute cutaneous lupus erythematosus, whereas chronic cutaneous lupus erythematosus was twice as common in adult systemic lupus erythematosus ( p < 0.001). The mean Systemic Lupus Erythematosus Disease Activity Index of juvenile systemic lupus erythematosus was significantly higher than that of adult systemic lupus erythematosus (14.29 ± 7.13 vs 11.27 ± 6.53). Multivariate analysis revealed the following associations in juvenile systemic lupus erythematosus: acute cutaneous lupus erythematosus and non-scarring alopecia with increased risk of arthralgia, mucosal ulcers with leukopenia, cutaneous vasculitis with seizure, and finding of granular casts. On the contrary, the associations for adult systemic lupus erythematosus were oral ulcers with arthralgia and cutaneous vasculitis with myositis. Conclusions Cutaneous signs in systemic lupus erythematosus may signal prognostic implication. Interestingly, despite similar cutaneous lesions in systemic lupus erythematosus, different ages of onset are associated with different systemic involvement.

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“…In addition, it may take several days or months before the classical pattern of joint involvement appear [ 9 ]. Characteristic clues such as malar and discoid rashes, photosensitivity, and oral ulcers could be absent at presentation [ 9 , 10 ]. Evidence of organ involvement such as raised creatinine, cytopenia, seizure, or psychosis may be found in close follow-up visits [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…Laboratory pitfalls are another challenge in the diagnosis of SLE in brucellosis-endemic regions. Considering the predisposition to exposure in endemic regions, a low-titer of serologic Brucella on the Wright and 2ME tests may be found [ 7 - 10 ]. Higher serologic titers indicate brucellosis, but low or intermediate titers are not able to differentiate SLE from brucellosis [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

Best Practice for Prolonged Fever in Primary Care Setting: Close Follow-Up or Empiric Antibiotic Therapy?

et al. 2018

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The management of prolonged fever in low-socioeconomic-status areas by primary care providers such as general practitioners is challenging. Given the endemic nature of many infectious diseases, physicians typically start empirical antibiotic therapy following a limited diagnostic workup including serologic examinations. Herein, we report the case of a young male patient with prolonged fever and arthralgia initially diagnosed with and treated for brucellosis but with a confirmed diagnosis of systemic lupus erythematosus on follow-up. This unique case shows that close follow-up is the best practice for managing prolonged fever in cases with non-specific laboratory findings.

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Prognostic implications of active discoid lupus erythematosus and malar rash at the time of diagnosis of systemic lupus erythematosus: Results from a prospective cohort study

Abstract: Malar rash may be a marker of more severe systemic disease over time, while DLE has no significant impact on general SLE disease activity.

Cited by 13 publications

References 16 publications

Cutaneous vasculitis in SLE

Cutaneous vasculitis in SLE

Skin signs in juvenile- and adult-onset systemic lupus erythematosus: clues to different systemic involvement

Best Practice for Prolonged Fever in Primary Care Setting: Close Follow-Up or Empiric Antibiotic Therapy?

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