Treatment of IgG4-related disease

Published on Jul 18, 2016in Zeitschrift Fur Rheumatologie0.90
· DOI :10.1007/s00393-016-0142-y
Cory A. Perugino5
Estimated H-index: 5
(Harvard University),
John H. Stone67
Estimated H-index: 67
(Harvard University)
IgG4-related disease (IgG4-RD) is capable of causing great morbidity and even mortality if the condition remains undiagnosed or poorly treated, yet is typically a treatment-responsive disorder. Glucocorticoids have not been studied rigorously and practices with regard to dosing and duration of treatment remain largely empiric. In addition, IgG4-RD patients are often particularly susceptible to and intolerant of the deleterious effects of glucocorticoid therapy. B cell depletion with anti-CD20 monoclonal antibodies appears to be a rapid, effective means of obtaining disease control and limiting patients’ glucocorticoid exposure, but this option is frequently not available. Other therapies targeting the B cell lineage may also be efficacious, and one is under study. The means by which depletion or inhibition of B cells and their progeny ameliorate IgG4-RD is coming into focus now through careful mechanistic studies of samples from treated patients. The mechanistic understanding of IgG4-RD will bring an array of specific targets for therapeutic intervention. Plasmablast-directed therapy with a CD19 monoclonal antibody is currently in clinical trials. CD4 + cytotoxic T lymphocytes and fibrosis, both observed nearly universally in the tissue of IgG4-RD patients, present two unexploited vulnerabilities in controlling and even reversing the effects of the disease. Further development of such therapies is a major goal of the next few years.
  • References (24)
  • Citations (9)
#1Arezou Khosroshahi (Emory University)H-Index: 25
#2Zachary S. Wallace (Harvard University)H-Index: 17
Last.Hiroshi Goto (TMU: Tokyo Medical University)H-Index: 24
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#1Mollie N. Carruthers (Harvard University)H-Index: 15
#2Topazian (Mayo Clinic)H-Index: 1
Last.John H. Stone (Harvard University)H-Index: 67
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#1Hamid Mattoo (Harvard University)H-Index: 16
#2Emanuel Della-Torre (Harvard University)H-Index: 13
Last.Shiv Pillai (Harvard University)H-Index: 45
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Cited By9
#1Chace Mitchell (UAB: University of Alabama at Birmingham)
#2Courtney B. Crayne (UAB: University of Alabama at Birmingham)H-Index: 2
Last.Randy Q. Cron (UAB: University of Alabama at Birmingham)H-Index: 42
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#1Chan Mi Lee (Cleveland Clinic Lerner College of Medicine)
#2Mohamed Alalwani (Cleveland Clinic)H-Index: 1
Last.Carmen Gota (Cleveland Clinic Lerner College of Medicine)H-Index: 7
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#1Mahmoud A. AbdelRazek (Harvard University)H-Index: 1
#2Nagagopal Venna (Harvard University)H-Index: 16
Last.John H. Stone (Harvard University)H-Index: 67
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#1Cory A. Perugino (MIT: Massachusetts Institute of Technology)H-Index: 5
#2Hamid Mattoo (MIT: Massachusetts Institute of Technology)H-Index: 16
Last.John H. Stone (Harvard University)H-Index: 67
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#1Bernardo BaptistaH-Index: 1
#2Alina Casian (Guy's and St Thomas' NHS Foundation Trust)H-Index: 6
Last.Claire M Rice (Southmead Hospital)H-Index: 2
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