Session Type: Abstract Submissions (ACR)
IgG4-related disease (IgG4-RD) is an immune-mediated multiorgan, fibroinflammatory disease often associated with an elevated serum IgG4 concentration. The diagnosis hinges on characteristic histopathologic features. Glucocorticoids (GC) are an effective but non-curative treatment with many known toxicities, and many patients relapse on low doses. We report here an experience with the use of B cell depletion in 58 patients with biopsy-proven IgG4-RD.
All 58 patients were treated and followed for at least three months in the Massachusetts General Hospital Center for IgG4-RD. Patients’ medical records were reviewed for details regarding demographics, clinical manifestations, prior treatment, response to treatment, and complications of treatment. The IgG4-RD Responder Index (IgG4-RD RI) was used to assess clinical improvement. Rituximab (RTX) (1gm) was administered on days 0 and 15. Three fourths of the patients in this cohort received no treatment except for RTX.
Fifty-eight patients were included. Their mean age was 56 years (range: 32-83). The mean number of organs involved was 2.2 (range: 1-6). Thirty-two patients (55%) had an elevated serum IgG4 concentration at baseline (mean 712 mg/dL; range 154-4780; normal < 135 mg/dL). Thirty-two (55%) of the patients had undergone treatment courses – GC in 24 (41%) – prior to treatment with B cell depletion. The mean duration of follow-up after the first RTX infusion was 597 days (range: 90-1770). Forty-three (74%) of the patients were treated with RTX alone.
Clinical improvement was observed in 88% of patients following RTX administration. Among the 25 patients with post-RTX imaging studies, 24 (96%) demonstrated either improvement (68%) or stability (28%) in the radiologic features. Among the 15 (26%) patients on GC at the time their RTX began, 11 (73%) were able to discontinue GC completely following RTX treatment and 4 (27%) were able to taper the dose to below 5 mg/day of prednisone. Among the patients with an elevated serum IgG4 concentration before RTX, the value declined to a mean of 248 mg/dl (range: 20-985) after RTX among the 29 patients with follow up values assessed; the value normalized in only 13 patients (44%).
Among 33 patients followed for more than one year, 18 (55%) experienced disease flares, an average of 10 months (range 5-27) after the first RTX infusion. Nineteen patients received more than one course of RTX (a total of 36 re-treatments, 24 for flares and 12 for remission maintenance). RTX was well tolerated; there were 15 adverse events among 13 patients. Infusions reactions (4) and infection (4) were the most common adverse events.
RTX appears to be an effective and well-tolerated treatment for IgG4-RD. The majority of patients treated with RTX require no concomitant GC therapy. Serum IgG4 concentrations improve but the majority do not normalize following RTX treatment, despite clinical improvement.
J. H. Stone,
Genentech and Roche,
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/rituximab-in-igg4-related-disease-a-large-single-center-experience/