Is rituximab effective for IgG4-related disease in the long term? Experience of cases treated with rituximab for 4 years

Abstract

A prospective open-label trial of rituximab (RTX) for IgG4-related disease (IgG4-RD) was recently described in the Annals of the Rheumatic Diseases by Carruthers et al.1 According to their results, RTX is effective as induction therapy for IgG4-RD without glucocorticoid in the short term. We agree and support their results. We have also prescribed RTX for typical cases of IgG4-RD, showing characteristics of younger age, experience of several relapses, no history of hepatitis B and, since 2011, hesitation to increase the dose of glucocorticoid due to complications. We are currently treating three cases using RTX. Our protocol is as follows. We prescribe 500 mg/body of RTX at the onset of relapse. Meanwhile, the dose of glucocorticoid is decreased as much as possible. The patients visit our hospital for blood tests once every few months. Whole-body CT is routinely performed once a year. When relapse is suspected, we examine the whole body by CT.The maximum period of follow-up since initial RTX is currently 4 years. Case 1 involved a 60-year-old Japanese woman. She presented with IgG4-related dacryoadenitis and sialadenitis, and autoimmune pancreatitis. The initial dose of glucocorticoid was 40 mg/day, but relapses repeated with the tapering of steroid doses. Immunosuppressants were added, but proved ineffective and a third relapse was experienced in 2011. RTX

Response to: ‘Is rituximab effective for IgG4-related disease in the long term? Experience of cases treated with rituximab for 4 years’ by Yamamotoet al

“…The efficacy of RTX observed by Yamamoto and colleagues1 is highly congruent with our own reported experience 2. The great majority of patients appear to respond swiftly and impressively to B cell depletion.…”

“…We thank Yamamoto and colleagues1 for their response to our paper2 and their description of their experience with rituximab (RTX) in IgG4-related disease (IgG4-RD) in three patients. Their letter raises a number of important points pertaining to the management of IgG4-RD, in general, and to the use of B-cell-depletion strategies, specifically.…”

Efficacy of abatacept for IgG4-related disease over 8 months

“…RTX initially led to immediate clinical remission, but the effective period of RTX gradually became shorter than that of the initial treatment. After the sixth administration of RTX, the patient presented with no effect 2. We performed re-biopsy of the lacrimal glands, which again showed abundant infiltration of IgG4-positive plasmacytes (the ratio of IgG4/IgG-positive cells: 70%) with fibrosis and a lot of germinal centres (figure 1G, H), but the translocation to malignant lymphoma was not observed.…”

“…We found that RTX is effective as an induction therapy for IgG4-RD in the short term, but that it is necessary to repeat the prescription of RTX. Furthermore, we had a patient who presented with resistance to RTX 2. After obtaining informed consent, we started the patient on abatacept (ABT) treatment.…”

Predicting therapeutic response in IgG4-related disease based on cluster analysis

“…It was found that glucocorticoid has efficacy in all patients with IgG4-RD, but most patients cannot discontinue steroid and require maintenance treatment [2,3]. Furthermore, relapse can easily occur with steroid tapering [4], and there are often patients who need immunosuppressants or biologic agents for disease control [5,6]. However, when we analyzed the patients enrolled in the SMART (Sapporo Medical University and related institutes database for investigation and best treatment for IgG4-RD) registry cohort [2], it was found that they were a fairly diverse group in their clinical features and laboratory data even though they met the comprehensive diagnostic criteria for IgG4-RD [7].…”