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  • Response to: ‘Is rituximab effective for IgG4-related disease in the long term? Experience of cases treated with rituximab for 4 years’ by Yamamoto et al

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Correspondence response
Response to: ‘Is rituximab effective for IgG4-related disease in the long term? Experience of cases treated with rituximab for 4 years’ by Yamamoto et al
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  1. John H Stone1,
  2. Mollie N Carruthers2,
  3. Mark D Topazian3,
  4. Arezou Khosroshahi4,
  5. Thomas E Witzig5,
  6. Zachary S Wallace6,
  7. Phillip A Hart3,
  8. Vikram Deshpande7,
  9. Thomas C Smyrk8,
  10. Suresh Chari3
  1. 1Allergy and Immunology Division, Department of Rheumatology, Massachusetts General Hospital, Boston, Massachusetts, USA
  2. 2Department of Rheumatology, Massachusetts General Hospital, Boston, Massachusetts, USA
  3. 3Department of Gastroenterology & Hepatology, Mayo Clinic, Rochester, Minnesota, USA
  4. 4Department of Rheumatology, Emory University School of Medicine, Atlanta, Georgia, USA
  5. 5Department of Hematology, Mayo Clinic, Rochester, Minnesota, USA
  6. 6Rheumatology Unit, Massachusetts General Hospital, Boston, Massachusetts, USA
  7. 7Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA
  8. 8Department of Pathology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr John H Stone, Allergy and Immunology Division, Department of Rheumatology, Massachusetts General Hospital, Boston, MA 02114, USA; jhstone{at}partners.org

https://doi.org/10.1136/annrheumdis-2015-207640

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    We thank Yamamoto and colleagues1 for their response to our paper2 and their description of their experience with rituximab (RTX) in IgG4-related disease (IgG4-RD) in three patients. Their letter raises a number of important points pertaining to the management of IgG4-RD, in general, and to the use of B-cell-depletion strategies, specifically.

    First, their patients are exemplary of the fact a sizeable subset of IgG4-RD patients has a propensity to disease relapse over time. This point has been underappreciated from early reports of the use of glucocorticoids to treat IgG4-RD because those reports were characterised by short follow-up periods. Although the great majority of patients respond to glucocorticoids initially, repeated disease flares are the rule for many.

    Second, patients with IgG4-RD endure substantial morbidity from the requirement for repeated courses (or continuous on treatment) with glucocorticoids. The three patients described by Yamamoto and colleagues are reported to have experienced bilateral avascular necrosis of the hips and glucose intolerance (severe diabetes mellitus in one case). Our general sense is that the toxicities of prolonged glucocorticoid use in this and other diseases are underestimated. Moreover, patients …

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