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. 2020 Apr;29(5):514-522.
doi: 10.1177/0961203320908932. Epub 2020 Feb 27.

Evolving phenotype of systemic lupus erythematosus in Caucasians: low incidence of lupus nephritis, high burden of neuropsychiatric disease and increased rates of late-onset lupus in the 'Attikon' cohort

D Nikolopoulos  1   2 , M Kostopoulou  3 , A Pieta  1 , T Karageorgas  1 , D Tseronis  1 , K Chavatza  1 , S Flouda  1 , P Rapsomaniki  1 , A Banos  1   2 , E Kremasmenou  4 , V Tzavara  5 , P Katsimbri  1 , A Fanouriakis  1   6 , D T Boumpas  1   2   7
Affiliations

Evolving phenotype of systemic lupus erythematosus in Caucasians: low incidence of lupus nephritis, high burden of neuropsychiatric disease and increased rates of late-onset lupus in the 'Attikon' cohort

D Nikolopoulos et al. Lupus. 2020 Apr.

Abstract

Objective: This study aimed to analyse the phenotype of systemic lupus erythematosus (SLE) at first presentation and during follow-up in a newly established SLE cohort based at 'Attikon' University Hospital. The hospital combines primary, secondary and tertiary care for the region of Western Attica, Greece.

Methods: This study comprised a mixed prevalent and incident cohort of 555 Caucasian patients diagnosed with SLE according to American College of Rheumatology 1997 criteria and/or the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) 2012 criteria. Demographic and clinical characteristics, patterns of severity, treatments and SLICC damage index were recorded for each patient at the time of diagnosis and at last evaluation.

Results: The mean age at lupus diagnosis was 38.3 years (standard deviation = 15.6 years), with a median disease duration at last follow-up of two years (interquartile range 1-11). At initial presentation, the most common 'classification' manifestations were arthritis (73.3%), acute cutaneous lupus (65%) and unexplained fever (25%), while among symptoms not included in any criteria set, Raynaud's phenomenon (33%) was the most common. Kidney and neuropsychiatric involvement as presenting manifestations were present in 10.3% and 11.5% cases, respectively. Irreversible damage accrual was present in 17.8% within six months of disease diagnosis, attributed mainly to thrombotic and neuropsychiatric disease. At last evaluation, 202 (36.4%) patients had developed severe disease, of whom more than half were treated with pulse cyclophosphamide.

Conclusion: In this cohort of Caucasian patients, lupus nephritis is not as common as in older cohorts, while neuropsychiatric disease is emerging as a major frontier in lupus prevention and care. These data may help to document changes in the natural history and treatment of SLE over time and may have implications for its early recognition and management.

Keywords: Prevalent cohort; damage; incident cohort; lupus criteria; non-lupus criteria.

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Figures

Figure 1
Figure 1
Immunological profile of subjects with SLE in the ‘Attikon’ cohort at diagnosis and cumulatively. SLE: systemic lupus erythematosus; LA: lupus anticoagulant; aPL: antiphospholipid antibodies.
Figure 2
Figure 2
Flow chart of all neuropsychiatric manifestations and types of events of the ‘Attikon’ cohort. Among 297 manifestations recorded, 127 were attributed to SLE, corresponding to 98 patients (17.6% of the whole cohort).
Figure 3
Figure 3
Types of treatment of subjects with SLE at both last evaluation and ever received in the ‘Attikon’ cohort. GCs: glucocorticoids; IV-MP: intravenous methylprednisolone; HCQ: hydroxychloroquine; IV-CYC: intravenous cyclophosphamide; MMF: mycophenolate; AZA: azathioprine; CsA: cyclosporine; MTX: methotrexate.
See this image and copyright information in PMC

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