American College of Rheumatology criteria at inception, and accrual over 5 years in the SLICC inception cohort.

OBJECTIVE: To determine the frequency of each American College of Rheumatology (ACR) criterion met at time of enrollment, and the increase in each of the criteria over 5 years. METHODS: In 2000 the Systemic Lupus International Collaborating Clinics (SLICC) recruited an international inception cohort of patients with systemic lupus erythematosus (SLE; ≥ 4 ACR criteria) who were followed at yearly intervals according to a standard protocol. Descriptive statistics were used to assess the total and cumulative number of ACR criteria met at each visit. Regression models were done to compare the increase of individual and cumulative criteria as a function of race/ethnicity group, and sex. RESULTS: In all, 768 patients have been followed for a minimum of 5 years. Overall, 59.1% of the patients had an increase in the number of ACR criteria they met over the 5-year period. The mean number of ACR criteria met at enrollment was 5.04 ± 1.13 and at year 5 was 6.03 ± 1.42. At enrollment, nonwhite patients had a higher number of ACR criteria (5.19 ± 1.23) than white patients. The total number of criteria increased in both white and nonwhite ethnicities, but increased more among whites. Males had a slightly lower number of criteria at enrollment compared to females and males accrued fewer criteria at 5 years. CONCLUSION: In this international inception cohort of SLE patients with at least 4 ACR criteria at entry, there was an accumulation of ACR criteria over the following 5 years. The distribution of criteria both at inception and over 5 years is affected by sex and ethnicity.

Assessment of coronary risk based on cumulative exposure to lipids in systemic lupus erythematosus.

OBJECTIVE: To quantify the independent role of each of low-density lipoprotein cholesterol (LDL-C), total cholesterol:high-density lipoprotein cholesterol ratio (TC:HDL-C), triglyceride (TG) level, and HDL-C as a marker of coronary risk in systemic lupus erythematosus (SLE). METHODS: Patients with lipid measurements taken before a coronary event (or last clinic visit) were included. Mean and time-adjusted mean (TAM) levels were calculated for each lipid variable in each patient. Time-dependent proportional hazards regression models were used to quantify the risk of coronary event [myocardial infarction (MI) or angina], after adjustment for age. RESULTS: Among 384 patients, over a mean (SD) followup of 3.81 (2.58) years, there were 21 "first" coronary events (6 MI, 15 angina). Mean and TAM LDL-C (HR 1.83, 95% CI 1.19-2.81, p = 0.006), TC:HDL ratio (HR 1.43, 95% CI 1.02-2.00, p = 0.04), and TG (HR 2.11, 95% CI 1.32-3.39, p = 0.0019) were predictive of coronary event at subsequent visits. In contingency table analysis, TAM LDL-C cutpoint of 2.0 mmol/l had a sensitivity and negative predictive value for coronary event of 85.7% (95% CI 63.7-97.0) and 93.9% (95% CI 83.1-98.7), respectively. However, at this cutpoint the specificity was only 12.7% (95% CI 9.4-16.5). CONCLUSION: This study links LDL-C, TC:HDL-C ratio, and TG to coronary risk in patients with SLE and quantifies the magnitude of this risk. SLE-specific risk assessment levels for lipids may be selected to optimize positive or negative predictive values.

Recommendations for frequency of visits to monitor systemic lupus erythematosus in asymptomatic patients: data from an observational cohort study.

OBJECTIVE: The aim of our study was to determine the optimal frequency of followup visits in patients with systemic lupus erythematosus (SLE). METHODS: Patients followed in the lupus clinic over a 2-year period who had at least 3 visits and at least 18 months of followup were included. At each visit patients undergo a complete history, physical examination, and laboratory evaluation. The following variables that would not have been recognized by the patient were identified: proteinuria, hematuria, pyuria, casts, low hemoglobin, leukopenia, thrombocytopenia, elevated serum creatinine, positive anti-DNA antibodies, and low complement. When one of these variables was detected, it was determined whether it was new, and whether other features of activity were present. Thus isolated new variables of interest were identified. Descriptive statistics were used. RESULTS: A total of 515 patients (89% female, 61% white) met the inclusion criteria, with an average of 6.1 ± 1.5 for a total of 3126 visits. The average length of time between visits was 3.8 ± 1.0 months. In the 515 patients, the variables of interest were the sole manifestation of SLE in 126 (24.5%) patients (in a total of 175 visits). The commonest manifestations were renal, low complement, and DNA antibodies followed by thrombocytopenia, low hemoglobin, and elevated creatinine. CONCLUSION: One in 4 patients with SLE seen over a 2-year period will have a solitary silent variable of interest that could be detected only by routine laboratory followup. Patients with mild or inactive disease should be followed with clinical and laboratory measures at 3-4 month intervals.

Clinically active serologically quiescent systemic lupus erythematosus.

OBJECTIVE: To identify the frequency and characteristics of clinical activity with serological quiescence (CASQ) in a large cohort of patients with systemic lupus erythematosus (SLE) followed prospectively at a single center. METHODS: Patients followed at the Lupus Clinic between 1991 and 1995 who on at least 3 consecutive visits had clinical activity in the absence of a low complement and elevated DNA binding were identified. Demographics, disease characteristics, and therapy for the CASQ periods, as well as prior and subsequent disease course until April 2002 were analyzed. RESULTS: Of 514 patients followed at the Lupus Clinic according to a standard protocol, 62 patients had at least one episode of CASQ lasting a 9.8 +/- 6.4 months. During these periods, patients showed evidence of clinical disease activity with a SLEDAI-2K of 8.9 +/- 5.3. Major organ involvement (central nervous system, renal, vasculitis) occurred in 43 patients. Forty-four patients were treated with prednisone 16.7 +/- 11.4 mg/day, 21 were on immunosuppressive medication and 30 on antimalarials. Of the 58 patients who had followup after their last CASQ defining visit, 9 remained CASQ for 39 +/- 23 months. Of the remaining 49 patients, 23 became inactive, 21 became clinically and serologically active, and 5 were serologically active but clinically quiescent (SACQ). CONCLUSION: Clinical laboratory correlation in SLE is a heterogeneous relationship. The majority of patients have clinical-serological concordance. The minority have discordance between clinical and serological status, and are either SACQ or CASQ. Therefore, monitoring both clinical and serological features in patients with SLE is important.