A. Raposo

Characterization of damage in Portuguese lupus patients: analysis of a national lupus registry.

Background: Although the survival rate has considerably improved, many patients with systemic lupus erythematosus (SLE) develop irreversible organ damage. Objectives: The objectives of this paper are to characterize cumulative damage in SLE patients and identify variables associated with its presence and severity. Methods: A cross-sectional analysis of SLE patients from the Portuguese Lupus register Reuma.pt/SLE in whom damage assessment using the SLICC/ACR-Disability Index (SDI) was available was performed. Predictor factors for damage, defined as SDI ≥ 1, were determined by logistic regression analyses. A sub-analysis of patients with severe damage (SDI ≥ 3) was also performed. Results: In total, 976 patients were included. SDI was ≥1 in 365 patients, of whom 89 had severe damage. Musculoskeletal (24.4%), neuropsychiatric (24.1%) and ocular (17.2%) domains were the most commonly affected. Older age, longer disease duration, renal involvement, presence of antiphospholipid antibodies and current therapy with steroids were independently associated with SDI ≥ 1. The subpopulation with severe damage had, in addition, a greater interval between the first manifestation attributable to SLE and the clinical diagnosis as well as and more frequently early retirement due to SLE. Conclusions: This large lupus cohort confirmed that demographic and clinical characteristics as well as medication are independently associated with damage. Additionally, premature retirement occurs more often in patients with SDI ≥ 3. Diagnosis delay might contribute to damage accrual.

AB0608 Greater Organ Involvement and Disease Activity in Childhood-Onset than Adult-Onset With SLE (DATA from Reuma.Pt/Les)

Background Systemic lupus erythematosus (SLE) is a multi-organ immune-mediated disease that affects predominantly women at reproductive age but may present itself at any age. Age at disease onset has a strong modulating effect on clinical presentation and course of disease. Although young patients may have a more aggressive disease, controversies persist regarding the impact of age at disease onset on SLE outcome. Objectives Characterize childhood-onset, adult-onset and late-onset SLE and assess whether disease outcome differs in these three patient groups. Methods Patients with childhood-onset (diagnosis ≤18 years) SLE fulfilling ACR 1997 criteria were identified in the Portuguese registry Reuma.pt/SLE and compared with adult-onset (≥19y and ≤49 years) and late-onset (≥50 years) SLE patients paired for disease duration. Results Two hundred and sixty seven SLE patients with mean disease duration of 11.9±9.3 years were analyzed (Table 1). The number of fulfilled ACR criteria was significantly higher in childhood-onset SLE. A greater proportion of women, higher prevalence of arthritis and anti-SSA antibodies were noted in the adult-onset group. Hypertension, diabetes and thyroid disease were significantly more prevalent in late-onset SLE. Disease activity at last visit evaluated using the SLEDAI-2K was significantly higher in childhood-onset group than in the late-onset counterparts. SLICC/ACR damage index was numerically higher in late-onset SLE and significantly more patients in this group had irreversible damage. Cyclophosphamide and mycophenolate mophetil were used more frequently in childhood-onset SLE patients.Table 1. Demographic and clinical characteristics of childhood-onset, adult-onset and late-onset SLE Childhood-onset Adult-onset Late-onset P N=89 N=89 N=89 Female n (%) 77 (87) 85 (96) 78 (88) 0.039 Current mean age (years) 25.1±9.1 40.0±13.1 68.3±7.3 <0.001 Number of ACR criteria fulfilled 5.8±1.3 5.3±1.3 5.2±1.1 0.005  Malar rash (%) 55 (62) 32 (36) 33 (37) <0.001  Arthritis (%) 62 (70) 79 (89) 71 (80) 0.005  Renal involvement (%) 52 (58) 28 (31) 14 (16) <0.001  Neurologic disorder (%) 10 (11) 5 (6) 2 (2) 0.039  Hematologic disorder (%) 68 (76) 53 (62) 67 (75) 0.029 Anti-SSA positivity (%) 13 (15) 30 (34) 19 (21) 0.006 Low complement (%) 74 (83) 60 (67) 46 (52) <0.001 SLEDAI-2K at last visit 3.4±3.8 2.2±2.7 1.6±2.8 0.004 SLICC-DI 0.5±0.9 0.7±1.3 0.9±1.3 0.116 SLICC-DI ≥1 (%) 18 (20) 23 (26) 36 (40) <0.001 Hypertension (%) 17 (19) 16 (18) 43 (48) <0.001 Diabetes (%) 0 (0) 7 (8) 15 (17) 0.008 Thyroid disease (%) 3 (3) 13 (26) 20 (23) 0.004 Conclusions The skin, kidney and neurological involvement are most common in childhood-onset, as well as the use of immunosuppressants, supporting the concept of a more severe disease. In contrast, patients with late-onset SLE have more comorbidities and irreversible damage. The age of SLE onset has a significant impact not only on the clinical characteristics and disease activity, but is also important for disease outcome. Disclosure of Interest None declared

FRI0428 The Weaker Sex: Characterization of Gender Disparities in A Nationwide Lupus Registry (REUMA.PT LES): Table 1.

Background Systemic lupus erythematosus (SLE) is characterized by female predominance with male to female ratio around 1:10. Differences regarding clinical manifestations, disease activity, damage and mortality between men and women with SLE have been reported. Overall it is recognized that gender may affect SLE phenotype, but results concerning disease severity and prognosis are still a matter of debate. Objectives Characterization of Portuguese SLE male patients, focusing demographic, clinical, and laboratorial features. Methods All SLE patients from the Portuguese Lupus Register, Reuma.pt/LES were included. Demographic, clinical and therapeutic data were analyzed upon records from the last visit. Student t-tests, chi-square tests and Fishers exact tests were used to compare male and female patients. Analyses were further adjusted to age and disease duration. Results Of the 1510 SLE patients registered in Reuma.pt/LES, 122 (8%) are men. Male patients had later onset (39.4±20.6y vs 35.6±14.1y; p=0.005) and shorter disease duration (10.7±7.6y vs 14.1±9.0 y; p=0.0001). Mean current age, racial distribution and education level was similar in the two groups. Serositis, renal involvement and hemolytic anemia were more prevalent in men while, photosensitivity, alopecia, oral ulcers and arthritis were more commonly found in women (Table 1). Thyroid disease was more frequent in women (11.4 vs 2.3%). Cardiovascular risk factors had a similar distribution between these groups. Accumulated damage assessed by the SLICC damage index (SDI) and disease activity, assessed by SLEDAI-2K at last visit were similar in the two groups, with adjustment to age and disease duration. Antimalarial drugs and steroids were used more frequently in women. Table 1. Characteristics of SLE in male and female patients Men (n=122) Women (n=1389) P Photosensitivity 36 (32.4) 620 (49.9) <0.001* Alopecia 7 (6.7) 310 (26.8) <0.001* Oral ulcers 20 (17.7) 395 (31.9) 0.008* Arthritis 65 (57.0) 906 (72.5) <0.001* Serositis 36 (32.1) 236 (18.9) 0.001* Renal Involvement 49 (44.1) 344 (28.1) <0.001* Neurologic disorder 6 (5.4) 59 (4.8) 0.448 Hemolytic anemia 18 (16.4) 122 (9.8) 0.031* Anti-dsDNA positivity 96 (84.96) 929 (74.7) 0.020 Anti-SSA positivity 15 (27.8) 688 (39.1) 0.064 SLEDAI-2K 2.3±3.0 2.6±3.1 0.650 SLICC 0.82±1.3 0.71±1.22 0.126 * Statistically significant differences, adjusted to age and disease duration. Conclusions Male patients with SLE are older at disease onset and present a distinct phenotype with less cutaneous, mucous membranes and articular manifestations. However, disease outcome evaluated by the SDI is comparable in men and women, which is in line with observations from other European cohorts. The acknowledgement of the effect of gender on disease manifestations may help physicians in the timely introduction of an appropriate care. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4615