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Kidney Week

Abstract: PO1891

Rituximab-Associated Flare of Cryoglobulinemic Vasculitis

Session Information

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Sy-Go, Janina Paula Tiulentino, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Thongprayoon, Charat, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Zoghby, Ziad, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Leung, Nelson, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Manohar, Sandhya, Mayo Clinic Minnesota, Rochester, Minnesota, United States
Background

Patients with cryoglobulinemic vasculitis (CV) can develop disease flare after rituximab administration. The pathogenesis is hypothesized to be from immune complex deposition in the microvasculature, wherein the immune complex consists of the involved cryoglobulin and an antigenic portion of rituximab. Our objective was to describe the prevalence, clinical characteristics, predisposing factors, and outcomes of rituximab-associated flare of CV.

Methods

We conducted a retrospective study in a tertiary referral center. We defined disease flare as any clinical deterioration within two weeks following rituximab administration, including onset of new organ involvement or worsening of the underlying CV not clearly explained by disease progression alone - with or without laboratory evidence.

Results

Among 64 patients with known CV who received rituximab therapy in our center, 14 (22%) developed disease flare. Median age was 67.5 years. Seven patients (50%) had type II CV while the other half had either type I (n=6) or type III (n=1). Twelve patients (86%) had IgM-mediated CV flare. Twelve patients (86%) had an underlying B-cell lymphoproliferative disorder as the cause of their CV. CV flare occurred after a median time of 5.5 days (range: 2-8 days). The organ systems most involved were the skin (n=10), kidneys (n=5), and peripheral nerves (n=3). Nearly all patients received treatment directed against their underlying diseases, including chemotherapy, corticosteroids, and/or plasmapheresis during the flare. Patients who developed flare were more likely to have B-cell lymphoproliferative disorder as the underlying etiology of their CV (p=0.03), had lower creatinine levels prior to rituximab treatment (0.8 vs. 1.05 mg/dL, p=0.01), and eventually received more treatments with plasmapheresis together with rituximab (p=0.03). Eight patients (57%) died after a median time of 27 months.

Conclusion

In our study, the prevalence of rituximab-associated flare of CV is 22%, and it can occur in all types of CV. Flares tend to arise about two days (less than one week) after rituximab administration and are more likely to happen in patients with an underlying B-cell lymphoproliferative disorder. These flares do not indicate failure of response to treatment. Clinicians should be cognizant of its existence and have a high index of suspicion for this phenomenon.