Rituximab for IgG4-related disease: A prospective, open-label trial

Mollie N. Carruthers, Mark Topazian, Arezou Khosroshahi, Thomas Elmer Witzig, Zachary S. Wallace, Philip A. Hart, Vikram Deshpande, Thomas Christopher Smyrk, Suresh T Chari, John H. Stone, Tore K. Kvien

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278 Scopus citations


Objectives: To evaluate the efficacy of rituximab (RTX) in IgG4-related disease (IgG4-RD) in an open-label pilot trial. Methods: We treated 30 IgG4-RD patients with two doses of RTX (1000 mg each). The participants were either treated with RTX alone (n = 26; 87%) or required to discontinue baseline glucocorticoids (GC) within 2 months (n = 4; 13%). Disease activity was measured by the IgG4-RD Responder Index (IgG4-RD RI) and physician's global assessment (PGA). Disease response was defined as the improvement of the IgG4-RD RI by two points. The primary outcome, measured at 6 months, was defined as: (1) decline of the IgG4-RD RI ≥2 points compared with baseline; (2) no disease flares before month 6; and (3) no GC use between months 2 and 6. Complete remission was defined as an IgG4-RD RI score of 0 with no GC use. Results: Disease responses occurred in 97% of participants. The baseline IgG4-RD RI and PGA values, 11±7 and 63±22 mm, respectively, declined to 1±2 and 11±16 mm at 6 months (both p<0.00001). The primary outcome was achieved by 23 participants (77%). Fourteen (47%) were in complete remission at 6 months, and 12 (40%) remained in complete remission at 12 months. Among the 19 with elevated baseline serum IgG4, IgG4 concentrations declined from a mean of 911 mg/dL (range 138-4780 mg/dL) to 422 mg/dL (range 56-2410 mg/dL) at month 6 (p<0.05). However, only 8 (42%) of the 19 achieved normal values. Conclusions: RTX appears to be an effective treatment for IgG4-RD, even without concomitant GC therapy.

Original languageEnglish (US)
Pages (from-to)1171-1177
Number of pages7
JournalAnnals of the Rheumatic Diseases
Issue number6
StatePublished - Jun 1 2015

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)
  • Immunology and Allergy

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    Carruthers, M. N., Topazian, M., Khosroshahi, A., Witzig, T. E., Wallace, Z. S., Hart, P. A., Deshpande, V., Smyrk, T. C., Chari, S. T., Stone, J. H., & Kvien, T. K. (2015). Rituximab for IgG4-related disease: A prospective, open-label trial. Annals of the Rheumatic Diseases, 74(6), 1171-1177. https://doi.org/10.1136/annrheumdis-2014-206605