Development of the American College of Rheumatology Patient-Reported Outcome Quality Measures for Systemic Lupus Erythematosus.

OBJECTIVE: As part of a Centers for Disease Control and Prevention-funded American College of Rheumatology (ACR) initiative, we sought to develop quality measures related to Patient Reported Outcome Measure (PROM) use for systemic lupus erythematosus (SLE) clinical care. METHODS: An expert workgroup composed of physician, patient, and researcher representatives convened to identify patient-reported outcome (PRO) domains of greatest importance to people with SLE. A patient advisory panel separately ranked domains. PROMs assessing priority domains were identified through structured literature review, and detailed psychometric reviews were conducted for each PROM. In a Delphi process, the expert workgroup rated PROMs on content validity, psychometric quality, feasibility of implementation, and importance for guiding patient self-management. The patient advisory panel reviewed PROMs in parallel and contributed to the final recommendations. RESULTS: Among relevant PRO domains, the workgroup and patient partners ranked depression, physical function, pain, cognition, and fatigue as high-priority domains. The workgroup recommended at least once yearly measurement for (1) assessment of depression using the Patient Health Questionnaire or Patient Reported Outcomes Measurement Information System (PROMIS) depression scales; (2) assessment of physical function using PROMIS physical function scales or the Multi-Dimensional Health Assessment Questionnaire; and (3) optional assessments of fatigue and cognition. Pain scales evaluated were not found to be sufficiently superior to what is already assessed in most SLE clinic visits. CONCLUSION: Expert workgroup members and patient partners recommend that clinicians assess depression and physical function at least once yearly in all people with SLE. Additional PROMs addressing cognition and fatigue can also be assessed. Next steps are to incorporate PROM-based quality measures into the ACR The Rheumatology Informatics System for Effectiveness registry.

Obexelimab in Systemic Lupus Erythematosus With Exploration of Response Based on Gene Pathway Co-Expression Patterns: A Double-Blind, Randomized, Placebo-Controlled, Phase 2 Trial.

OBJECTIVE: Obexelimab is an investigational, bifunctional, noncytolytic monoclonal antibody that binds CD19 and FcyRIIb to inhibit B cells, plasmablasts, and plasma cells. This trial evaluated the efficacy and safety of obexelimab in the treatment of patients with systemic lupus erythematosus (SLE). METHODS: During screening, patients with active, non-organ-threatening SLE received corticosteroid injections to ameliorate symptoms while immunosuppressants were withdrawn (≤10 mg/day prednisone equivalent and ≤400 mg/day hydroxychloroquine allowed). Patients with improved disease activity were randomized 1:1 to obexelimab 5 mg/kg intravenously or placebo once every 2 weeks until week 32 or loss of improvement (LOI). RESULTS: In this study, 104 patients were randomized. Analysis of the primary endpoint, proportion of patients reaching week 32 without LOI, used an efficacy-evaluable (EE) population defined as patients who completed the study or withdrew for flare or treatment-related toxicity. This endpoint did not reach statistical significance: 21 of 50 obexelimab-treated patients (42.0%) versus 12 of 42 patients (28.6%) treated with a placebo (P = 0.183). Time to LOI was increased in obexelimab-treated patients versus patients treated with a placebo in the EE (hazard ratio [HR] 0.53, P = 0.025) and intention-to-treat (HR 0.59, P = 0.062) populations. In obexelimab-treated patients, B cells decreased approximately 50%, and trough concentration and inclusion in baseline gene expression clusters with high B cell pathway modules were associated with increased time to LOI. Obexelimab was associated with infusion reactions but was generally safe and well-tolerated. CONCLUSION: Although the primary endpoint was not reached, secondary analysis showed time to LOI was significantly increased in obexelimab-treated patients, and analysis of patient subsets defined by gene expression patterns at baseline suggests a responding subpopulation.

A Case of Acute Necrotizing Myopathy in a Patient with Systemic Lupus Erythematosus.

BACKGROUND Systemic lupus erythematosus (SLE) is an autoimmune disorder affecting multiple organ systems with a wide spectrum of clinical presentation and associated with positive serologies. Musculoskeletal involvement in patients with SLE is relatively uncommon, occurring in approximately 4% to 16% of cases. Some patients can develop necrotizing myopathy without myositis. MRI in patients with SLE-associated necrotizing myopathy usually shows interstitial edema, while muscle biopsy often shows type 2 muscle fiber atrophy. We herein report an unusual case of acute necrotizing myopathy in a patient recently diagnosed with SLE. This case report focuses on the pertinent features related to the diagnosis of this patient while highlighting the management of acute necrotizing myopathy. CASE REPORT A 30-year-old African American woman presented to the Emergency Department with skin rashes, myalgia, polyarthralgia, and muscle weakness resulting in the inability to walk, 2 weeks after being diagnosed with SLE. Laboratory analysis showed elevated creatine kinase and myoglobin. She was found to have both MRI and biopsy findings suggestive of necrotizing myopathy. She was treated with mycophenolate mofetil and steroids, with an improvement of muscle strength and decrease in creatine kinase over a 2-week period. CONCLUSIONS Immune-mediated necrotizing myopathies are a rare group of debilitating myopathies that can be associated with SLE. The diagnosis of necrotizing myopathy in patients with SLE requires a high index of suspicion and careful work-up to establish a diagnosis. Muscle biopsy often shows type 2 muscle fiber atrophy. Immunosuppressive therapy is the mainstay of treatment, and early initiation of immunotherapies is associated with an improvement in patient outcomes.

Hydroxychloroquine in patients with systemic lupus erythematosus with end-stage renal disease.

OBJECTIVES: To determine dosing patterns and examine predictors of filled hydroxychloroquine (HCQ) prescriptions in patients with systemic lupus erythematosus (SLE) with end-stage renal disease (ESRD). METHODS: This was a retrospective cohort study of patients with SLE in the US Renal Data System (USRDS) database in fiscal year 2011. All patients were Medicare Part D beneficiaries. Patients with a diagnosis of SLE were identified by the International Classification of Diseases, 9th revision code 710. The prevalence, dosing, and predictors of filled HCQ prescriptions (demographic factors, dialysis type, and provider subspecialty) were determined. RESULTS: There were 10,276 patients with SLE identified; 2048 (19.9%) had a prescription for HCQ filled. The mean daily dose of HCQ was 321 mg (range 58-2000 mg). The most common daily doses were 200 (n=768, 37.5%) and 400 mg (n=1161, 56.7%). In multivariable logistic regression analysis, significant predictors of filled HCQ prescriptions included black/African-American race (OR 1.34, 95% CI (1.17 to 1.46)), hemodialysis (1.50, 95% CI (1.29 to 1.74)), and care from a rheumatologist (5.06, 95% CI (4.56 to 5.62)). Negative predictors of filled HCQ prescriptions included male gender (OR 0.72, 95% CI (0.63 to 0.83)) and those aged 45 years or older (compared to 20 years old and younger, aged 45-64 years, OR 0.66, 95% CI (0.54 to 0.79); aged 65-74 years, OR 0.58, 95% CI (0.44 to 0.76); aged 75 years and older, OR 0.56, 95% CI (0.39 to 0.82)). CONCLUSIONS: In patients with SLE with ESRD, the dosing strategies for HCQ with regard to potential toxicity and disparities in prescribing patterns need further study.