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

Abstract

ObjectiveChanges in the care of patients with SLE dictate a re-evaluation of its natural history and risk factors for disease deterioration and damage accrual. We sought to decipher factors predictive of a deterioration in phenotype (‘transition’) in patients initially presenting with non-severe disease.MethodsPatients from the ‘Attikon’ cohort with disease duration ≥1 year were included. Disease at diagnosis was categorised as mild, moderate or severe, based on the British Isles Lupus Assessment Group manifestations and physician judgement. ‘Transition’ in severity was defined as an increase in category of severity at any time from diagnosis to last follow-up. Multivariable logistic regression was performed to identify baseline factors associated with this transition.Results462 patients were followed for a median (IQR) of 36 (120) months. At diagnosis, more than half (56.5%) had a mild phenotype. During disease course, transition to more severe forms was seen in 44.2%, resulting in comparable distribution among severity patterns at last follow-up (mild 28.4%, moderate 33.1%, severe 38.5%). Neuropsychiatric involvement at onset (OR 6.33, 95% CI 1.22 to 32.67), male sex (OR 4.53, 95% CI 1.23 to 16.60) and longer disease duration (OR 1.09 per 1 year, 95% CI 1.04 to 1.14) were independently associated with transition from mild or moderate to severe disease. Patients with disease duration ≥3 years who progressed to more severe disease had more than 20-fold increased risk to accrue irreversible damage.ConclusionAlmost half of patients with initially non-severe disease progress to more severe forms of SLE, especially men and patients with positive anti-double-stranded DNA or neuropsychiatric involvement at onset. These data may have implications for the management of milder forms of lupus.

Update οn the diagnosis and management of systemic lupus erythematosus

“…Although most patients with SLE initially present with mild disease, a proportion may progress in severity, so that mild, moderate and severe cases are equally split over time to one-third in each category. Data from Nikolopoulos et al 6…”

Demyelinating Syndromes in Systemic Lupus Erythematosus: Data From the “Attikon” Lupus Cohort

“…The “Attikon” lupus cohort was established in 2015 in the Rheumatology Unit of the “Attikon” University Hospital, Athens, serving as a referral center for patients with lupus, as previously described ( 6 , 7 ). As of December 2021, it includes 708 Caucasian SLE patients.…”

A Modern Approach to the Diagnosis and Management of Systemic Lupus Erythematosus

“…Однако со временем болезнь прогрессирует, так что число пациентов с легкой, средней и тяжелой степенями активности заболевания становится приблизительно одинаковым (около трети в каждой категории) (рис. 3) [12]. the disease, classification criteria are improved constantly.…”

“…Fig.3. Intensity of systemic lupus erythematosus at onset and with progression of the disease[12].Note. The first column in each group shows the rate of intensity of a certain disease at onset; the second column is intensity with progression of the diseaseТерапия.…”