Journal ArticleDOI
IgG4‐related disease with hypergammaglobulinemic hyperviscosity and retinopathy
Patrick Wong,Adrian T. Fung,Alina S. Gerrie,Gregory Moloney,David Maberley,David W. Rossman,Valerie A. White,David R. Collins,Robert Coupland,Luke Y.C. Chen +9 more
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TLDR
A novel case of IgG4‐RD with hypergammaglobulinemic hyperviscosity responsive to fludarabine and rituximab is described, which can be considered in patients with severe refractory disease.Citations
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Journal ArticleDOI
Immunology of IgG4‐related disease
TL;DR: The most recent insights on the immunology of IgG4‐RD are reviewed, focusing on the relative contribution of innate and adaptive immune responses to the full pathological phenotype of this fibrotic condition.
Journal ArticleDOI
IgG4-related disease: what a hematologist needs to know.
TL;DR: This review focuses on the hematologic manifestations of IgG4-related disease, including lymphadenopathy, eosinophilia, and polyclonal hypergammaglobulinemia, and targeted therapy against plasmablasts, IgE and other disease biomarkers warrant further exploration.
Journal ArticleDOI
IgG4- related disease: an orphan disease with many faces
TL;DR: Current knowledge on clinical manifestations, pathophysiology and treatment of IgG4-RD is summarized, although adjunctive immunosuppressive agents are used in relapsing patients.
Journal ArticleDOI
Acute hyperviscosity: syndromes and management.
TL;DR: Whole-blood hyperviscosity is seen in patients with extreme elevation of the red cell and white cell count, and plasma exchange is the therapy of choice and is relatively safe.
Journal ArticleDOI
T helper 2 and regulatory T-cell cytokine production by mast cells: a key factor in the pathogenesis of IgG4-related disease.
Mai Takeuchi,Yasuharu Sato,Kyotaro Ohno,Satoshi Tanaka,Katsuyoshi Takata,Yuka Gion,Yorihisa Orita,Toshihiro Ito,Tomoyasu Tachibana,Tadashi Yoshino +9 more
TL;DR: Real-time polymerase chain reaction analysis confirmed the significant upregulation of interleukin (IL)4, IL10, and transforming growth factor beta 1 (TGFβ1) in IgG4-related disease and indicated the presence of mast cells expressing these cytokines in diseased tissues.
References
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Journal ArticleDOI
Consensus statement on the pathology of IgG4-related disease
Vikram Deshpande,Yoh Zen,John Kc Chan,Eunhee E Yi,Yasuharu Sato,Tadashi Yoshino,Günter Klöppel,J. Godfrey Heathcote,Arezou Khosroshahi,Judith A. Ferry,Rob C. Aalberse,Daniel Bloch,William R. Brugge,Adrian C Bateman,Mollie N. Carruthers,Suresh T. Chari,Wah Cheuk,Lynn D. Cornell,Carlos Fernandez-del Castillo,David G. Forcione,Daniel L. Hamilos,Terumi Kamisawa,Satomi Kasashima,Shigeyuki Kawa,Mitsuhiro Kawano,Gregory Y. Lauwers,Yasufumi Masaki,Yasuni Nakanuma,Kenji Notohara,Kazuichi Okazaki,Ji Kon Ryu,Takako Saeki,Dushyant V. Sahani,Thomas C. Smyrk,James Robert Stone,Masayuki Takahira,George Webster,Motohisa Yamamoto,Giuseppe Zamboni,Hisanori Umehara,John H. Stone +40 more
TL;DR: This statement proposes a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy, and advocates the use of strict criteria for accepting newly proposed entities or sites as components of the IgG 4- related disease spectrum.
Journal ArticleDOI
Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease.
TL;DR: Treatment with rituximab led to prompt clinical and serologic improvement in these patients with refractory IgG4-RSD, and is a viable treatment option for this condition, suggesting that ritUXimab achieves its effects in IgG 4- RSD by depleting the pool of B lymphocytes that replenish short-lived IgG3-secreting plasma cells.
Journal ArticleDOI
Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients.
Arezou Khosroshahi,Mollie N. Carruthers,Vikram Deshpande,Sebastian Unizony,Daniel Bloch,John H. Stone +5 more
TL;DR: Treatment with RTX led to prompt clinical and serologic improvement in refractory IgG4-RD in all patients with active inflammation, and Serial treatments with RTX may lead to progressive declines in serum IgG 4 concentrations and better disease control.
Journal ArticleDOI
IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity.
Wah Cheuk,John K. Chan +1 more
TL;DR: An elevated serum titer of immunoglobulin G4 (IgG4), the least common of the 4 subclasses of IgG, is a surrogate marker for the recently characterized IgG4-related sclerosing disease, which affects predominantly middle-aged and elderly patients, with male predominance.
Related Papers (5)
Consensus statement on the pathology of IgG4-related disease
Vikram Deshpande,Yoh Zen,John Kc Chan,Eunhee E Yi,Yasuharu Sato,Tadashi Yoshino,Günter Klöppel,J. Godfrey Heathcote,Arezou Khosroshahi,Judith A. Ferry,Rob C. Aalberse,Daniel Bloch,William R. Brugge,Adrian C Bateman,Mollie N. Carruthers,Suresh T. Chari,Wah Cheuk,Lynn D. Cornell,Carlos Fernandez-del Castillo,David G. Forcione,Daniel L. Hamilos,Terumi Kamisawa,Satomi Kasashima,Shigeyuki Kawa,Mitsuhiro Kawano,Gregory Y. Lauwers,Yasufumi Masaki,Yasuni Nakanuma,Kenji Notohara,Kazuichi Okazaki,Ji Kon Ryu,Takako Saeki,Dushyant V. Sahani,Thomas C. Smyrk,James Robert Stone,Masayuki Takahira,George Webster,Motohisa Yamamoto,Giuseppe Zamboni,Hisanori Umehara,John H. Stone +40 more
Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011
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