Treatment Algorithms in Systemic Lupus Erythematosus.

OBJECTIVE: To establish agreement on systemic lupus erythematosus (SLE) treatment. METHODS: SLE experts (n = 69) were e-mailed scenarios and indicated preferred treatments. Algorithms were constructed and agreement determined (≥50% respondents indicating ≥70% agreement). RESULTS: Initially, 54% (n = 37) responded suggesting treatment for scenarios; 13 experts rated agreement with scenarios. Fourteen of 16 scenarios had agreement as follows: discoid lupus: first-line therapy was topical agents and hydroxychloroquine and/or glucocorticoids then azathioprine and subsequently mycophenolate (mofetil); uncomplicated cutaneous vasculitis: initial treatment was glucocorticoids ± hydroxychloroquine ± methotrexate, followed by azathioprine or mycophenolate and then cyclophosphamide; arthritis: initial therapy was hydroxychloroquine and/or glucocorticoids, then methotrexate and subsequently rituximab; pericarditis: first-line therapy was nonsteroidal antiinflammatory drugs, then glucocorticoids with/without hydroxychloroquine, then azathioprine, mycophenolate, or methotrexate and finally belimumab or rituximab, and/or a pericardial window; interstitial lung disease/alveolitis: induction was glucocorticoids and mycophenolate or cyclophosphamide, then rituximab or intravenous gamma globulin (IVIG), and maintenance followed with azathioprine or mycophenolate; pulmonary hypertension: glucocorticoids and mycophenolate or cyclophosphamide and an endothelin receptor antagonist were initial therapies, subsequent treatments were phosphodiesterase-5 inhibitors and then prostanoids and rituximab; antiphospholipid antibody syndrome: standard anticoagulation with/without hydroxychloroquine, then a thrombin inhibitor for venous thrombosis, versus adding aspirin or platelet inhibition drugs for arterial events; mononeuritis multiplex and central nervous system vasculitis: first-line therapy was glucocorticoids and cyclophosphamide followed by maintenance with azathioprine or mycophenolate, and then rituximab, IVIG, or plasmapheresis; and serious lupus nephritis: first-line therapy was glucocorticoids and mycophenolate, then cyclophosphamide then rituximab. CONCLUSION: We established variable agreement on treatment approaches. For some treatment decisions there was good agreement between experts even if no randomized controlled trial data were available.

Systemic lupus international collaborating clinics renal activity/response exercise: comparison of agreement in rating renal response.

OBJECTIVE: To assess the degree to which physicians agree with each other and with ratings obtained with 3 existing responder indices, in rating the response to treatment of lupus nephritis. METHODS: Lupus nephritis patient medical records from 125 pairs of visits (6 months apart) were used to create renal response scenarios. Seven nephrologists and 22 rheumatologists rated each scenario as demonstrating complete response, partial response, same, or worsening. The plurality (most frequent) rating of renal response by the physicians was compared with the calculated score from the renal component of the British Isles Lupus Assessment Group (BILAG) index (original and updated [2004] version) and of the Responder Index for Lupus Erythematosus (RIFLE). The degree of agreement among the physicians was assessed by calculating intraclass correlation coefficients (ICCs). The degree of agreement between the plurality physician rating and ratings obtained with the established response indices was assessed using the kappa statistic. RESULTS: The ICC among all physicians was 0.64 (0.62 for nephrologists and 0.67 for rheumatologists). The chance-adjusted measure of agreement (kappa coefficient) between the plurality physician rating and the calculated score obtained using established indexes was 0.50 (95% confidence interval [95% CI] 0.38-0.61) for the RIFLE, 0.14 (95% CI 0.03-0.25) for the original BILAG, and 0.23 (95% CI 0.21-0.44) for the BILAG 2004. CONCLUSION: These findings indicate that rheumatologists as a group and nephrologists as a group have equal agreement in their rating of renal response. There was moderate agreement between plurality physician ratings and ratings obtained using the renal component of the RIFLE. Ratings of response using an index based on the original BILAG did not have good agreement with the plurality physician rating.

Systemic lupus international collaborating clinics renal activity/response exercise: development of a renal activity score and renal response index.

OBJECTIVE: To develop a measure of renal activity in systemic lupus erythematosus and use it to develop a renal response index. METHODS: Abstracted data from the medical records of 215 patients with lupus nephritis were sent to 8 nephrologists and 29 rheumatologists for rating. Seven nephrologists and 22 rheumatologists completed the ratings. Each physician rated each patient visit with respect to renal disease activity (none, mild, moderate, or severe). Using the most commonly selected rating for each patient as the gold standard, stepwise regression modeling was performed to identify the variables most related to renal disease activity, and these variables were then used to create an activity score. This activity score could then be applied to 2 consecutive visits to define a renal response index. RESULTS: The renal activity score was computed as follows: proteinuria 0.5-1 gm/day (3 points), proteinuria 0.5-1 gm/day = 3 points, proteinuria >1-3 gm/day = 5 points, proteinuria >3 gm/day = 11 points, [corrected] urine red blood cell count > = 5/hpf = 3 points, [corrected] urine white blood cell count > or = 5/hpf = 1 point. [corrected] The chance-adjusted agreement between the renal response index derived from the activity score applied to the paired visits and the plurality physician response rating was 0.69 (95% confidence interval 0.59-0.79). CONCLUSION: Ratings derived from this index for rating of renal response showed reasonable agreement with physician ratings in a pilot study. The index will require further refinement, testing, and validation. A data-driven approach to create renal activity and renal response indices will be useful in both clinical care and research settings.

Cancer screening in patients with systemic lupus erythematosus.

OBJECTIVE: To examine whether patients with systemic lupus erythematosus (SLE) undergo cancer screening according to established guidelines, to compare their reported screening practices with information from the general population, and to examine potential predictors of screening within our SLE sample. METHODS: We conducted a patient survey of cancer screening practices within the Montreal General Hospital lupus cohort. We compared self-reported frequency of cancer screening to guidelines suggested for the general population, and to figures for cancer screening reported in the general population. We also developed logistic regression models to establish potential predictors of screening for patients with SLE, with cervical cancer screening being the outcome of interest in our primary analyses. RESULTS: Of 48 women aged 50-69, 53% (95% confidence interval, CI: 38-68) had had a mammogram in the past 12 months, compared to 74% (95% CI: 73-75) for similarly aged Quebec women. Of 51 subjects aged 50 and older, only 18% (95% CI: 8-34) reported screening (fecal occult blood check with or without endoscopy) within the recommended time frame, compared to 48% (95% CI: 45-51) for colorectal screening for persons > 50 in the general population. Only 9 of 27 patients with SLE aged less than 30 had Pap tests in the past 12 months (33%, 95% CI: 19-52), compared with a general population rate of 56% (95% CI: 53-59) for similarly aged Quebec women. Our logistic regression model suggested that, among the SLE patients, non-whites, those with lower education, and those with higher disease damage scores were less likely to undergo cervical Pap testing. CONCLUSION: These data suggest that appropriate cancer screening may be overlooked in patients with SLE.