Objective. To describe the 6-month clinical outcome and the long-term safety profile of B cell depletion therapy (BCDT) in 50 patients with active systemic lupus erythematosus (SLE), who were nonresponsive or poorly responsive to conventional immunosuppression. Methods. All except 4 of 50 patients with active SLE received 1 gm of rituximab, 750 mg of cyclophosphamide, and 100 -250 mg of methylprednisolone, administered on 2 occasions 2 weeks apart, to achieve B cell depletion. Clinical outcome was assessed using the British Isles Lupus Assessment Group (BILAG) activity index and serial serologic measurements of disease activity. Remission was defined as a change from a BILAG A or B score to a C or D score in every organ system. Partial remission was a change from a BILAG A or B score to a C or D score in at least 1 system, but with the persistence of 1 score of A or B in another system. No improvement was defined as a BILAG A or B score that remained unchanged after treatment. Results. Of the 45 patients available for followup at 6 months, 19 patients (42%) achieved remission, and 21 patients (47%) reached partial remission after 1 cycle of BCDT (mean followup 39.6 months). BCDT resulted in a decrease in median global BILAG scores from 12 to 5 (P < 0.0001) and median anti-double-stranded DNA antibody titers from 106 to 42 IU/ml (P < 0.0001), and an increase in the median C3 level from 0.81 to 0.95 mg/liter (P < 0.02) at 6 months. Five serious adverse events were observed. Conclusion. BCDT is an effective treatment for patients with active SLE whose disease has failed to respond to standard immunosuppressive therapy. Although the safety profile of BCDT is favorable, ongoing monitoring is required.

Objectives: To describe the long-term clinical outcome and safety profile of B cell depletion therapy (BCDT) in patients with systemic lupus erythematosus (SLE). It was also determined whether baseline parameters can predict the likelihood of disease flare. Methods: 32 patients with refractory SLE were treated with BCDT using a combination protocol (rituximab and cyclo-phosphamide). Patients were assessed with the British Isles Lupus Assessment Group (BILAG) activity index, and baseline serology was measured. Flare was defined as a new BILAG ‘A’ or two new subsequent ‘B’s in any organ system. Results: Of the 32 patients, 12 have remained well after one cycle of BCDT (median follow-up 39 months). BCDT was followed by a decrease of median global BILAG scores from 13 to 5 at 6 months (p = 0.006). Baseline anti-extractable nuclear antigen (ENA) was the only identified independent predictor of flare post-BCDT (p = 0.034, odds ratio = 8, 95% CI 1.2 to 55) from multivariable analysis. Patients with low baseline serum C3 had a shorter time to flare post-BCDT (p = 0.008). Four serious adverse events were observed. Conclusion: Autoantibody profiling may help identify patients who will have a more sustained response. Although the long-term safety profile of BCDT is favourable, ongoing vigilance is recommended.

A minority of patients with SLE are SACQ. The majority of these patients experience a flare in the next 5 years and close followup is recommended. Anti-NCS antibodies may be a better predictor than anti-dsDNA antibodies for future flares.

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This study aims to report the concomitant diseases observed and damage outcome in a cohort of patients with adult idiopathic inflammatory myositis (IIM) during long-term follow-up. All patients with IIM were identified from a single centre (follow-up between 1979 and 2006) and fulfilled at least three of the four Bohan and Peter criteria. Patients with inclusion body myositis, juvenile-onset myositis and overt overlap syndromes were excluded. Medical notes were retrospectively reviewed. Concomitant diseases identified were divided into 12 different organ systems (bone, cardiac, respiratory, gastrointestinal, renal, central nervous, malignancy, infection, endocrine, eyes, dermatological and haematological). Patient damage index was calculated using the Myositis Damage Index tool. Fifty-five patients (31 polymyositis, 24 dermatomyositis) were identified. The most prevalent organ system involved was lung with 40 events per 1,000 patient years follow-up. There was significant steroid-related complications with 17/18 patients with bone involvement having osteopenia/osteoporosis. Sjogren's syndrome (n = 3) was the most frequent concomitant auto-immune disease observed. Patients with a higher number of organ systems involved had a significantly higher damage index (r = 0.48, p = 0.001). White patients showed a significant trend to develop more than three other organ system involvement (p < 0.0001) and myositis-related lung disease (p < 0.0001) compared to other races. There is significant steroid-related morbidity in adult IIM patients under long-term follow-up. The prevalence of another concomitant auto-immune disease unlike patients with lupus or Sjogren's syndrome is low.