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

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.

Update οn the diagnosis and management of systemic lupus erythematosus

“…Key disease features and their frequency at disease onset and cumulatively can be found in figure 3. 18 Diagnosis can be challenging in (1) early stages of the disease, when a limited number of features may be present; (2) antinuclear antibody (ANA)negative cases or organ-dominant forms and (3) rare disease presentations, which can nonetheless be severe and require prompt treatment. In our experience, non-rheumatologists fail to look consistently for arthritis and to take into consideration features of the disease not present simultaneously.…”

“…Newer sets of classification criteria [21][22][23] enable the earlier classification of SLE, with the combination of all three sets (ACR-1997, SLICC-2012 and EULAR/ACR-2019) ensuring the capturing of non-overlapping groups of patients (although at the expense of reduced specificity). 18 ANA or other immunologic positivity (autoantibodies or hypocomplementemia) are required for classification of SLE according to the SLICC-2012 and EULAR/ACR-2019, but not the ACR-1997 criteria. Fulfilment of the classification criteria is not necessary for the diagnosis for SLE.…”

“…Childhood SLE has higher activity at presentation, is more likely to be severe and to receive more aggressive therapy, as well as accumulate damage. Data from Nikolopoulos et al 18 26 27…”

Autoimmune encephalitis with psychiatric features in adults: historical evolution and prospective challenge

“…The differential diagnosis of autoimmune encephalitis is often demanding, and other diseases must be carefully ruled out in every patient. Several other disease entities need to be considered, such as the neuropsychiatric phenotype of lupus erythematosus often associated with double-stranded deoxyribonucleic acid antibodies, anti-antiphospholipid antibodies, anti-ß2-glycoprotein-I, lupus anti-coagulant or anti-ribonucleoprotein antibodies (Nikolopoulos et al 2020 ). Furthermore, Hashimoto encephalopathy in conjunction with psychiatric symptoms and thyroid autoimmunity must be excluded by thoroughly investigating the existence of antibodies against thyroid peroxidase (TPO) and thyroglobulin (TG) (Barbero et al 2019 ; Barbuti et al 2017 ).…”

Transition to severe phenotype in systemic lupus erythematosus initially presenting with non-severe disease: implications for the management of early disease

“…The 'Attikon' lupus cohort consists of a 'prevalent cohort' (patients with an SLE diagnosis prior to the establishment of the patient registry) and an 'inception' cohort (patients followed from diagnosis onwards). 12 A standardised data set, including demographics and clinical and laboratory features of the disease, is completed for each patient at first visit and every follow-up. All immunosuppressive/immunomodulatory drugs administered for the treatment of SLE are also documented, including current treatment (ie, at most recent visit) and past medications.…”