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Natural history of disease activity and damage in patients with cutaneous lupus erythematosus.

ABSTRACT: BACKGROUND:Long-term studies characterizing disease course of cutaneous lupus erythematosus (CLE) patients on standard-of-care treatments are lacking. OBJECTIVE:We characterized and compared disease course of CLE patients using Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). METHODS:In total, 83 CLE patients with CLASI scores collected from ?3 study visits within 2 years had disease activity and damage trends calculated by average change scores (ACS). Trends were classified as improved (ACS ?-3), worsened (ACS ?3), or stable (-3 < ACS < 3). Linear regression models compared CLASI trends between groups. RESULTS:Most patients (72.73%) with initial CLASI activity (CLASI-A) scores >9 (N = 33) had improved disease activity versus 14.00% of those with initial CLASI-A scores ?9 (N = 50). Linear regression analyses showed significant improvement in CLASI-A scores in patients of minority races (P < .05), with baseline CLASI-A scores >9 (P < .0001), baseline CLASI damage (CLASI-D) scores ?10 (P = .0001), and CLE disease duration ?1 year (P = .01). Of 28 patients with baseline CLASI-D scores ?10, 35.71% had improvements in damage, while 5.26% of patients with initial CLASI-D scores of 5-9 (N = 19) and 0% with initial CLASI-D scores <5 (N = 36) (P = .0005) had improvements. LIMITATIONS:Limitations include small sample size. CONCLUSION:Baseline CLASI-A score >9, minority race, and short disease duration predict CLE disease activity improvement. A baseline CLASI-D score ?10 is associated with disease damage improvement.

SUBMITTER: Ker KJ

PROVIDER: S-EPMC6234101 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature

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Natural history of disease activity and damage in patients with cutaneous lupus erythematosus.

Ker Khor Jia KJ Teske Noelle M NM Feng Rui R Chong Benjamin F BF Werth Victoria P VP

Journal of the American Academy of Dermatology 20180630 6

<h4>Background</h4>Long-term studies characterizing disease course of cutaneous lupus erythematosus (CLE) patients on standard-of-care treatments are lacking.<h4>Objective</h4>We characterized and compared disease course of CLE patients using Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI).<h4>Methods</h4>In total, 83 CLE patients with CLASI scores collected from ≥3 study visits within 2 years had disease activity and damage trends calculated by average change scores (ACS). ...[more]

PMID: 29969701

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Project description:INTRODUCTION:Systemic lupus erythematosus (SLE) is characterized by frequent neuropsychiatric involvement, which includes cognitive impairment (CI). We aimed at assessing CI in a cohort of Italian SLE patients by using a wide range of neurocognitive tests specifically designed to evaluate the fronto-subcortical dysfunction. Furthermore, we aimed at testing whether CI in SLE is associated with serum autoantibodies, disease activity and chronic damage. METHODS:Fifty-eight consecutive patients were enrolled. Study protocol included data collection, evaluation of serum levels of ANA, anti-dsDNA, anti-cardiolipin, anti-?(2)-glycoprotein I, anti-P ribosomal, anti-endothelial cell, and anti-Nedd5 antibodies. SLEDAI-2000 and SLICC were used to assess disease activity and chronic damage. Patients were administered a test battery specifically designed to detect fronto-subcortical dysfunction across five domains: memory, attention, abstract reasoning, executive function and visuospatial function. For each patient, the raw scores from each test were compared with published norms, then transformed into Z scores (deviation from normal mean), and finally summed in the Global Cognitive Dysfunction score (GCDs). RESULTS:Nineteen percent of patients had mild GCDs impairment (GCDs 2-3), 7% moderate (GCDs 4-5) and 5% severe (GCDs?6). The visuospatial domain was the most compromised (MDZs?=?-0.89±1.23). Anti-cardiolipin IgM levels were associated with visuospatial domain impairment (r?=?0.331, P?=?0.005). SLEDAI correlated with GCDs, and attentional and executive domains; SLICC correlated with GCDs, and with visuospatial and attentional domains impairment. CONCLUSIONS:Anti-phospholipids, disease activity, and chronic damage are associated with cognitive dysfunction in SLE. The use of a wide spectrum of tests allowed for a better selection of the relevant factors involved in SLE cognitive dysfunction, and standardized neuropsychological testing methods should be used for routine assessment of SLE patients.

Project description:Objective:The severity and disease course of cutaneous lupus erythematosus (CLE) are highly variable. Consequently, outcome measures for CLE clinical improvement are heterogeneous, complicating treatment decisions and therapeutic development. This study characterises CLE outcome measures and identifies the influence of clinical improvement thresholds on strengths of associations with patient demographic and clinical factors. Methods:In this pilot cohort study, multivariable models identified factors associated with CLE activity and skin damage improvement, defined as relative decreases in Cutaneous Lupus Activity and Severity Index (CLASI) activity (CLASI-A) and damage (CLASI-D) scores, over ranges of response thresholds. Results:66 patients with 119 visit-pairs were included in the CLASI-A analysis. 74 patients with 177 visit-pairs were included in the CLASI-D analysis. Factors associated with CLE activity and damage improvement depended on the response threshold. Some associations were stronger at more stringent thresholds, including subacute CLE predominance with increased likelihood of CLASI-A improvement (R 2=0.73; 50% reduction: OR 1.724 (95% CI 0.537 to 5.536); 75%: 5.67 (95% CI 1.56 to 20.5)) and African-American race with decreased likelihood of CLASI-D improvement (R 2=0.80; 20%: 0.40 (95% CI 0.17 to 0.93); 40%: 0.25 (95% CI 0.08 to 0.82)). Other associations were stable across multiple thresholds, including older age of CLE development with increased likelihood of CLASI-A improvement (R 2=0.25; 50%: 1.05 (95% CI 1.01 to 1.09]; 75%: 1.05 (95% CI 1.00 to 1.10)) and higher initial disease activity with decreased likelihood of CLASI-D improvement (R 2=0.55; 20%: 0.91 (95% CI 0.84 to 0.98); 40%: 0.88 (95% CI 0.79 to 0.97)). Conclusions:Examining a range of CLASI threshold outcomes can comprehensively characterise changes in disease course in patients with CLE. Insufficiently stringent thresholds may fail to distinguish meaningful clinical change from natural fluctuation in disease activity.

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Natural history of disease activity and damage in patients with cutaneous lupus erythematosus.

Publications

Natural history of disease activity and damage in patients with cutaneous lupus erythematosus.

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