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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Abstract

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
Volume74
Issue number6
DOIs
StatePublished - Jun 1 2015

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Labels
Immunoglobulin G
Glucocorticoids
Rituximab
Physicians
Reference Values
Therapeutics

ASJC Scopus subject areas

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

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Rituximab for IgG4-related disease : A prospective, open-label trial. / Carruthers, Mollie N.; Topazian, Mark; Khosroshahi, Arezou; Witzig, Thomas Elmer; Wallace, Zachary S.; Hart, Philip A.; Deshpande, Vikram; Smyrk, Thomas Christopher; Chari, Suresh T; Stone, John H.; Kvien, Tore K.

In: Annals of the Rheumatic Diseases, Vol. 74, No. 6, 01.06.2015, p. 1171-1177.

Research output: Contribution to journalArticle

Carruthers, MN, Topazian, M, Khosroshahi, A, Witzig, TE, Wallace, ZS, Hart, PA, Deshpande, V, Smyrk, TC, Chari, ST, Stone, JH & Kvien, TK 2015, 'Rituximab for IgG4-related disease: A prospective, open-label trial', Annals of the Rheumatic Diseases, vol. 74, no. 6, pp. 1171-1177. https://doi.org/10.1136/annrheumdis-2014-206605
Carruthers, Mollie N. ; Topazian, Mark ; Khosroshahi, Arezou ; Witzig, Thomas Elmer ; Wallace, Zachary S. ; Hart, Philip A. ; Deshpande, Vikram ; Smyrk, Thomas Christopher ; Chari, Suresh T ; Stone, John H. ; Kvien, Tore K. / Rituximab for IgG4-related disease : A prospective, open-label trial. In: Annals of the Rheumatic Diseases. 2015 ; Vol. 74, No. 6. pp. 1171-1177.
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abstract = "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.",
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T2 - A prospective, open-label trial

AU - Carruthers, Mollie N.

AU - Topazian, Mark

AU - Khosroshahi, Arezou

AU - Witzig, Thomas Elmer

AU - Wallace, Zachary S.

AU - Hart, Philip A.

AU - Deshpande, Vikram

AU - Smyrk, Thomas Christopher

AU - Chari, Suresh T

AU - Stone, John H.

AU - Kvien, Tore K.

PY - 2015/6/1

Y1 - 2015/6/1

N2 - 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.

AB - 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.

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