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Extended report
Rituximab for IgG4-related disease: a prospective, open-label trial
  1. Mollie N Carruthers1,
  2. Mark D Topazian2,
  3. Arezou Khosroshahi3,
  4. Thomas E Witzig4,
  5. Zachary S Wallace1,
  6. Philip A Hart2,
  7. Vikram Deshpande5,
  8. Thomas C Smyrk6,
  9. Suresh Chari2,
  10. John H Stone1
  1. 1Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA
  2. 2Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA
  3. 3Division of Rheumatology, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA
  4. 4Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA
  5. 5Department of Pathology, Mayo Clinic, Rochester, Minnesota, USA
  6. 6Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr John H Stone, Rheumatology Unit, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA; jhstone{at}


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.

Trial registration number identifier: NCT01584388.

  • B cells
  • Inflammation
  • Treatment

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