1 Konstantinos, et al: Monophasic disease in SLE Personal non-commercial use only. The Journal of Rheumatology Copyright © 2018. All rights reserved. Monophasic Disease Course in Systemic Lupus Erythematosus Konstantinos Tselios, Dafna D. Gladman, Zahi Touma, Jiandong Su, Nicole Anderson, and Murray B. Urowitz ABSTRACT. Objective. Disease course in systemic lupus erythematosus (SLE) is primarily relapsing-remitting. Long quiescent and chronically active patterns are less frequent. We recently described an atypical “monophasic” course in a small number of patients. The aim of the present study was to assess the prevalence and characteristics of such patients in a defined SLE cohort. Methods. The inception patients of the University of Toronto Lupus Clinic (enrolled within 18 mos of diagnosis) were investigated. No time interval > 18 months was allowed between consecutive visits. A monophasic course was defined as Systemic Lupus Erythematosus Disease Activity Index 2000 = 0 (serology excluded), achieved within 5 years since enrollment and maintained for ≥ 10 years. Descriptive statistics were used. Results. Of 267 inception patients, 27 (10.1%) achieved prolonged clinical remission (≥ 10 yrs) and 20 (7.5%) sustained remission for the entire followup (18 yrs on average). Twelve patients were receiving no maintenance treatment 10 years after achieving remission. Clinical manifestations at diagnosis (apart from skin and musculoskeletal involvement) included 25% in each of central nervous system involvement and lupus nephritis (LN). Half the patients were serologically active. Ten years after achieving remission, two-thirds of the patients had discontinued glucocorticosteroids; the remaining were treated with 5 mg/day on average. Seven patients relapsed after 10 years, 4 with arthritis, 2 LN, and 1 catastrophic antiphospholipid syndrome. Conclusion. A monophasic disease course was observed in 7.5% in this inception cohort. Patients sustained remission for 18 years on average, eventually without medications. Further study of such patients may provide unique pathophysiologic insights for SLE. (J Rheumatol First Release June 1 2018; doi:10.3899/jrheum.171319) Key Indexing Terms: SYSTEMIC LUPUS ERYTHEMATOSUS PROLONGED REMISSION MONOPHASIC DISEASE From the Centre for Prognosis Studies in Rheumatic Diseases, Toronto Lupus Clinic, University Health Network, Toronto, Ontario, Canada. The University of Toronto Lupus Research Program is supported by Lou and Marissa Rocca, Lupus Canada, and the Canadian Institute of Health Research [Competition No 201610PJT, Application No. 377979]. K. Tselios, MD, PhD, Centre for Prognosis Studies in Rheumatic Diseases, Toronto Lupus Clinic, University Health Network; D.D. Gladman, MD, FRCPC, Centre for Prognosis Studies in Rheumatic Diseases, Toronto Lupus Clinic, University Health Network; Z. Touma, MD, FACP, Centre for Prognosis Studies in Rheumatic Diseases, Toronto Lupus Clinic, University Health Network; J. Su, MB, MSc, Centre for Prognosis Studies in Rheumatic Diseases, Toronto Lupus Clinic, University Health Network; N. Anderson, HBSc, CCRP, Centre for Prognosis Studies in Rheumatic Diseases, Toronto Lupus Clinic, University Health Network; M.B. Urowitz, MD, FRCPC, Centre for Prognosis Studies in Rheumatic Diseases, Toronto Lupus Clinic, University Health Network. Address correspondence to Dr. M.B. Urowitz, Centre for Prognosis Studies in the Rheumatic Diseases, Toronto Western Hospital, 399 Bathurst St., 1E-410B, Toronto, Ontario M5T 2S8, Canada. E-mail: m.urowitz@utoronto.ca Accepted for publication February 28, 2018. Systemic lupus erythematosus (SLE) is primarily a relap- sing-remitting disease with unpredictable flares interspersed with periods of clinical quiescence of varying duration. Initial studies from the Hopkins Lupus Cohort on the patterns of disease activity over time described 3 different subgroups of patients: long quiescent, relapsing-remitting, and chronically active in 4.5 years of followup 1 . Chronically active disease was the most common pattern, accounting for 58% and 40% of the cumulative patient-years, as evaluated by the physician’s global assessment (PGA) and the modified Systemic Lupus Erythematosus Disease Activity Index (SLEDAI; excluding serology), respectively 1 . A more recent study from the same center, where all patients with at least 1 year of followup were included, yielded different results applying the same definitions 2 . Relapsing-remitting disease was the most prevalent pattern (54% and 50%, as assessed by the PGA and modified SLEDAI, respectively), followed by long quiescence (31% by the modified SLEDAI) 2 . Steiman, et al described an unusual course of “mono- phasic” disease in a small subset of patients (11/1613, 0.7%) who achieved prolonged remission 3 . In that study, the proportion of patients who achieved a state of complete remission for > 5 years without medications reached 2.4% of the entire cohort. The monophasic patients sustained complete remission for an average of 11.5 years, eventually without medications. Although these studies provided valuable insights into the www.jrheum.org Downloaded on January 21, 2022 from