Background/Purpose: Disease course in systemic lupus erythematosus (SLE) is believed to follow three distinct patterns over time, namely relapsing-remitting, persistently active and prolonged quiescent. We recently showed that a small subgroup of patients might follow a course characterized by an initial active clinical presentation, followed by complete clinical remission, occasionally with no medications. The aim of the present study was to assess the prevalence, demographic, clinical and immunological characteristics of such patients.

Methods: The inception cohort a large lupus clinic (patients enrolled within 18 months from diagnosis) was investigated. Selected patients had a minimum follow-up of 10 years and no interval greater than 18 months between consecutive visits. Prolonged clinical remission was defined as clinical SLEDAI-2K=0 (serology excluded), achieved within the first five years since diagnosis and maintained for ≥10 years. Descriptive statistics were used.

Results: Of 883 inception patients, 382 met inclusion criteria. Twenty-seven (24 females, 21 Caucasians) achieved prolonged clinical remission (prevalence 7.1%) in 1.6 ± 1.3 years (median 1.2) since diagnosis. Mean age at diagnosis was 39±14.1 years. Clinical manifestations at diagnosis included mucocutaneous involvement in 19 (70.4%), arthritis in 16 (59.3%), serositis in 5 (18.5%), nephritis in 7 (25.9%), central nervous system in 2 (7.4%) and cytopenias in 15 (55.6%). Immunological and therapeutic characteristics are shown in Table 1.

Cumulative glucocorticosteroid dose over 10 years was 18.1±12.4grams. Beyond 10 years, 7 patients (25.9%) relapsed at a mean of 15.2±3.8 years since diagnosis (four with musculoskeletal and/or skin involvement). In the remaining three patients (two with nephritis and one with catastrophic antiphospholipid syndrome), the flare occurred during or shortly after pregnancy. Twenty of the 27 patients (5.2% of the entire cohort) sustained remission for the entire length of follow-up (17.8±6.7 years) and thus had pure monophasic disease. Twelve patients (44.4%) were not taking any medications after 10 years. Three patients died (11.1%), one from lung cancer and two from unknown cause.

Conclusion: A monophasic disease course was observed in 5.2% of our inception SLE patients, while approximately half of them maintained remission without any medications after 10 years from diagnosis.