@article {Nikolopoulose000394, author = {Dionysis S Nikolopoulos and Myrto Kostopoulou and Antigoni Pieta and Sofia Flouda and Katerina Chavatza and Aggelos Banos and John Boletis and Pelagia Katsimbri and Dimitrios T Boumpas and Antonis Fanouriakis}, title = {Transition to severe phenotype in systemic lupus erythematosus initially presenting with non-severe disease: implications for the management of early disease}, volume = {7}, number = {1}, elocation-id = {e000394}, year = {2020}, doi = {10.1136/lupus-2020-000394}, publisher = {Archives of Disease in childhood}, abstract = {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 ({\textquoteleft}transition{\textquoteright}) in patients initially presenting with non-severe disease.Methods Patients from the {\textquoteleft}Attikon{\textquoteright} 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. {\textquoteleft}Transition{\textquoteright} 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.}, URL = {https://lupus.bmj.com/content/7/1/e000394}, eprint = {https://lupus.bmj.com/content/7/1/e000394.full.pdf}, journal = {Lupus Science \& Medicine} }