Background We sought to analyze the phenotype of the disease at the time of presentation and at last follow-up, in a newly established SLE cohort in Attica based at ‘Attikon’ University Hospital which combines primary, secondary and tertiary care for the region.

Methods In this retrospective study, 555 Caucasian patients were included diagnosed with SLE according to ACR 1997 criteria and/or the SLICC 20112 criteria. Demographic data, clinical course, serology, treatments, severity pattern and SLICC damage index (SDI) were recorded for each patient at the time of diagnosis and at last evaluation.

Results Mean (SD) age at lupus diagnosis was 38.3 years (±15.6) with median (IQR) disease duration at last follow-up 2 (10) years. Irreversible damage accrual was prevalent in 17.8% of lupus individuals at diagnosis, involving mainly thrombotic and neuropsychiatric events. At initial presentation, most common criteria manifestations were arthritis (73.3%), acute cutaneous lupus (65%) and leukopenia (23.8%), while among symptoms not included in any criteria set, Raynaud’s phenomenon (33%) and unexplained fever (25%) were most prevalent. Renal and neuropsychiatric involvement as presenting manifestations were present at 10.3% and 11.5%, respectively. Clinical characteristics at the time of diagnosis and cumulatively, are summarized in table 1. At the time of diagnosis, 6.3% of patients were ANA negative, whereas only one third had positive anti-dsDNA. At last evaluation, 202 patients (36.4%) had severe lupus and more than half patients were treated with pulses of cyclophosphamide.

Conclusions In this cohort of Caucasian patients, lupus nephritis is not as common as indicated in older literature, while neuropsychiatric disease is an emerging frontier in lupus prevention and care. These data may have implications for early recognition and diagnosis of SLE.