Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose
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.
Methods
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.
Results
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.
Conclusion
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.
Disclosure:
Z. Wallace,
None;
M. Carruthers,
None;
J. H. Stone,
Genentech and Roche,
5.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/rituximab-in-igg4-related-disease-a-large-single-center-experience/