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OBJECTIVE: To determine the frequency of prolonged remission in systemic lupus erythematosus (SLE) using strict criteria for remission and to define disease characteristics and prognosis of patients achieving this state. To also determine the frequency of remission utilizing less restrictive definitions, such as allowing shorter period of disease quiescence, persistence of serological activity, or treatment in the absence of clinical disease. METHODS: Patients registered in the Lupus Clinic database between 1970 and 1997 with visits no more than 18 months apart were identified. Prolonged remission was defined as a 5-year consecutive period of no disease activity (SLE disease activity index, SLEDAI = 0) and without treatment (corticosteroids, antimalarials, or immunosuppressants). Prolonged serologically active, clinically quiescent (SACQ) was defined as active serology (elevated anti-dsDNA by Farr assay or hypocomplementemia) but no clinical activity on SLEDAI and no treatment. RESULTS: Seven hundred and three patients fulfilled inclusion criteria. Of the 703 patients 46 (6.5%) achieved complete remission for at least 1 year, whereas only 12 patients (1.7%) had prolonged complete remission of at least 5 years on no treatment. Although the frequency of disease manifestations was similar to the patients not in remission, the 5-year remission group was distinguished by lower overall disease activity as measured by adjusted mean SLEDAI, lower prevalence of anti-DNA antibodies, and lower use of steroids and antimalarials. CONCLUSION: Prolonged complete remission in lupus is rare. Therefore with current therapies continued vigilance for disease recurrence is necessary.