The chronic damage in systemic lupus erythematosus is driven by flares, glucocorticoids and antiphospholipid antibodies: results from a monocentric cohort.

OBJECTIVES: Literature data suggest a significantly higher mortality in patients affected by systemic lupus erythematosus (SLE) developing chronic damage. Therefore, damage prevention is a major goal in the management of SLE patients. In the present study, we assessed damage by means of the Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) damage index (SDI), in a large cohort of SLE patients. Additionally, we aimed at evaluating its association with demographic and clinical features as well as with disease activity and laboratory findings. PATIENTS AND METHODS: We enrolled consecutive patients affected by SLE diagnosed according to the American College of Rheumatology (ACR) 1997 revised criteria. Chronic damage was determined by SDI calculated at the last examination in all patients with at least six months of follow-up. Disease activity was assessed by the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K); flare was defined as an increase of SLEDAI-2K ≥ 4 compared with the previous visit. RESULTS: We evaluated 349 SLE patients (M/F 25/324, mean age ± SD 42.7 ± 12.4 years, mean disease duration ± SD 164.9 ± 105.2 months). Among the enrolled patients, 125 (35.8%) showed a SDI ≥ 1 (mean SDI ± SD 1.7 ± 0.9, range 0-5). The musculo-skeletal was the most frequently involved organ/system in SDI score (41/349 patients, 11.7%), with deforming/erosive arthritis in 21/349 (6.0%). The presence of chronic damage was associated with age (P < 0.001), disease duration (P < 0.001), number of flares (P = 0.02) and with the use of glucocorticoids (P = 0.02). The logistic regression analysis revealed the association between neuropsychiatric damage and antiphospholipid syndrome (P = 0.01, OR = 3.9) and between the presence of cardiovascular damage and anti-ß2GPI antibodies (P = 0.01, OR 6.2). CONCLUSIONS: In the present study chronic damage was identified in about one third of SLE patients. The association between SDI and the number of flares claim for a thigh-control of the disease activity in order to prevent the chronic damage. The possible role of antiphospholipid antibodies (aPL) in the development of neuropsychiatric and cardiovascular damage may suggest a more careful assessment of such aPL positive patients.

Validation of a disease-specific health-related quality of life measure in adult Italian patients with systemic lupus erythematosus: LupusQoL-IT.

OBJECTIVE: The objective of this paper is to assess the validity of a linguistically validated version of the Lupus Quality of Life (LupusQoL(©)) in Italian patients affected by systemic lupus erythematosus (SLE). METHODS: Consecutive SLE patients completed the Italian version of the LupusQoL(©) and the Short Form (SF)-36. Disease activity was evaluated by the SLE disease activity Index-2000 (SLEDAI-2 K), and chronic damage by the Systemic Lupus International Collaborating Clinics/American College Rheumatology (ACR) Damage Index score (SDI). Internal consistency and test-retest reliability, convergent and discriminant validity were examined. Factor analysis with varimax rotation was performed. RESULTS: A total of 117 Italian SLE patients (M:F 13:104; mean age 40.6 ± 11.6 years, mean disease duration 127.5 ± 94.1 months) were recruited into the study. The Italian version of the LupusQoL(©) demonstrated substantial evidence of convergent validity in these patients when compared with equivalent items of the SF-36. In addition, the LupusQoL(©) discriminated between patients with different degrees of disease activity as measured by the SLEDAI-2 K. SLE patients with higher disease activity (SLEDAI-2K ≥4) showed poor QoL compared with those with lower disease activity (SLEDAI-2K <4), with significant differences in the domains of physical health, planning, burden to others and fatigue (p = 0.001, p = 0.04, p = 0.03, p = 0.04, respectively). The confirmatory factor analysis using the eight domain loadings of the 34 items showed a poor fit (χ(2)/degree of freedom (df) 2.26, χ(2 )= 1128.6 (p < 0.001), root mean square error of approximation (RMSEA) = 0.167; goodness-of-fit index (GFI) = 0.606, comparative fit index (CFI) = 0.649)). Screeplot analysis suggested a five-factor loading structure and confirmatory factor analysis result of which is similar to the eight-factor model. A good internal consistency was observed (Cronbach's α 0.89-0.91). Test-retest reliability was good to excellent between baseline and day 15 (intraclass correlation coefficient (ICC) 0.90-0.98). CONCLUSION: The Italian version of the LupusQoL(©) is a valid tool for adult patients with SLE.