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

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

Research output: Contribution to journalArticlepeer-review

316 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

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • General Biochemistry, Genetics and Molecular Biology


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