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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.

SUBMITTER: Nikolopoulos D

PROVIDER: S-EPMC7168806 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature

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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.

Nikolopoulos D D Kostopoulou M M Pieta A A Karageorgas T T Tseronis D D Chavatza K K Flouda S S Rapsomaniki P P Banos A A Kremasmenou E E Tzavara V V Katsimbri P P Fanouriakis A A Boumpas D T DT

Lupus 20200227 5

<h4>Objective</h4>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.<h4>Methods</h4>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/o ...[more]

PMID: 32106788

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Project description:BackgroundArguments still exist on prognosis of late-onset SLE, especially their kidney function. The purpose of this study was to investigate long-term kidney outcomes in patients with late-onset lupus nephritis (LN).MethodsA retrospective long-term cohort study was conducted in adult Chinese patients with LN. The patients were divided into late- (>50 years) and early-onset (<50 years) LN groups. The baseline characteristics, especially the kidney pathological characteristics, were compared. The cohort was followed-up for kidney outcome defined as doubling of serum creatinine or ESRD. Cox regression analysis was used to examine the association between late onset LN and its outcomes.ResultsA total of 1,264 patients were recruited, who were assigned to late-onset LN with 102 patients and early-onset LN with 1,162 patients. The late-onset LN group showed a worse baseline kidney function and more chronic pathological lesions than the early-onset LN group. During a follow-up time of 55 (3, 207) months, 114 (13.1%) deaths occurred, 107 (12.2%) had doubling of creatinine, and 80 (9.1%) developed end-stage kidney disease. The 5- and 10-year survival rates of the late-onset LN group were 67.6 and 50.5%, respectively, which were much worse than those of the early-onset LN group (89.8 and 84.6%, respectively). However, no significant difference was found on kidney survival (log-rank chi-square = 3.55, p = 0.06). Cox regression analysis showed that late-onset LN was an independent risk factor for patient survival (hazard ratio = 3.03, 95% CI (1.39, 6.58), p = 0.005). Increased baseline serum creatinine was an independent risk factor for kidney survival of patients with late-onset LN.ConclusionsPatients with late-onset LN had milder active lesions but severer chronic lesions in kidney pathology. They have poorer overall outcome but relatively favorable kidney outcome.Trial registrationClinicalTrials.gov Identifier: NCT03001973, 22 December 2016 retrospectively registered.

Project description:Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease that frequently affects the kidneys, known as lupus nephritis (LN). Such patients are treated with antimalarials, corticosteroids or immunosuppressive drugs, and more recently, target-specific biological drugs. Although efficacy of these therapies improved SLE-related outcomes, SLE remains associated with higher rates of infections. Here, we performed a comprehensive systemic review of infectious complications in clinical trials covering drug interventions for SLE or specifically for active LN. Our search in 15 online registries yielded a total of 1477 studies of which 14 matched our prespecified criteria. These covered the biological drugs anifrolumab, belimumab, and rituximab that were tested in patients with non-renal SLE and active LN.The available safety data from the SLE trials indicated that infectious complications such as herpes zoster, upper respiratory tract infection, nasopharyngitis, bronchitis, and urinary tract infection in patients receiving placebo were quite prevalent especially in the EXPLORER (rituximab) trial. Infections occurred mostly during the first year of LN therapy. Serious adverse events and infectious complications occurred more frequently in placebo-treated patients with active LN, especially in the BLISS-LN (belimumab) and LUNAR (rituximab) trials. Anifrolumab and rituximab increased the number of clinically relevant episodes of herpes zoster compared to belimumab in patients with active LN. Anifrolumab displayed a similar trend for influenza infections, which is consistent with the specific mechanisms-of-action of anifrolumab; highlighting drug-specific effects on infectious complications. In addition, standard-of-care therapy, e.g., MMF and immunosuppressants, as well as a longer SLE duration may also affect the incidence of serious adverse events and certain infectious complications in SLE patients with active LN.Infectious complications are common in SLE but even more common in patients with active LN, especially herpes zoster is strongly associated with active LN and anifrolumab therapy (OR 2.8, 95% CI 1.18 to 6.66, p = 0.018). Immunotherapy seems to impose unspecific and specific risks for infections. The latter may imply specific precautions such as preemptive vaccination and individual risk-benefit assessments.

Dataset Information

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.

Publications

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.

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