RT Journal Article
SR Electronic
T1 Transition to severe phenotype in systemic lupus erythematosus initially presenting with non-severe disease: implications for the management of early disease
JF Lupus Science & Medicine
JO Lupus Sci Med
FD Lupus Foundation of America
SP e000394
DO 10.1136/lupus-2020-000394
VO 7
IS 1
A1 Dionysis S Nikolopoulos
A1 Myrto Kostopoulou
A1 Antigoni Pieta
A1 Sofia Flouda
A1 Katerina Chavatza
A1 Aggelos Banos
A1 John Boletis
A1 Pelagia Katsimbri
A1 Dimitrios T Boumpas
A1 Antonis Fanouriakis
YR 2020
UL http://lupus.bmj.com/content/7/1/e000394.abstract
AB Objective Changes 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.Methods Patients 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.Results 462 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.Conclusion Almost 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.