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Dupilumab as a novel steroid-sparing treatment for IgG4-related disease.

Published on Apr 1, 2020in Annals of the Rheumatic Diseases14.299
· DOI :10.1136/ANNRHEUMDIS-2019-216368
Rachel Simpson1
Estimated H-index: 1
,
Stephanie Ka Ching Lau1
Estimated H-index: 1
(U of O: University of Ottawa)
+ 0 AuthorsJason Kihyuk Lee1
Estimated H-index: 1
Abstract
IgG4-related disease (IgG4-RD) is a rare fibroinflammatory, multisystemic condition with a relapsing-remitting progression.1 The level of serum IgG4 correlates with inflammatory activity and organ involvement.1 Glucocorticoids are first line for IgG4-RD, but there are numerous adverse effects with chronic use.2 Dupilumab is a monoclonal antibody that acts on the interleukin 4 (IL-4) receptor alpha, shared by the IL-4 and IL-13 receptors.1 IL-4 causes isotype switching from IgM to IgG4 and IL-13 is implicated in fibrosis.3 Thus, it was postulated by the authors to investigate dupilumab as a novel steroid-sparing treatment for IgG4-RD. A 67-year-old man with no known allergies and a history of sensory neural hearing loss, recurrent bronchitis, spinal stenosis, moderate positional obstructive sleep apnoea, asthma, atopic dermatitis (which caused swelling around his eyes) and allergic rhinoconjunctivitis underwent extensive investigations over the past 2 years due to suspected IgG4-RD. The patient’s initial complaint was pruritic erythematous lesions on the legs, …
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  • Citations (7)
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References6
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#1Mirei Shirakashi (Kyoto University)H-Index: 1
#2Hajime Yoshifuji (Kyoto University)H-Index: 21
Last. Mimori Tsuneyo (Kyoto University)H-Index: 54
view all 25 authors...
Glucocorticoids (GC) are effective for treating IgG4-related disease (IgG4-RD); however, relapse is often observed. We conducted a retrospective multicentre study to investigate risk factors in GC regimens associated with relapses of IgG4-RD. Data on 166 patients with definitive IgG4-RD diagnosis were collected from 12 institutions. Comprehensive surveillance of clinical backgrounds and GC regimens as well as multivariate analysis of factors associated with treatment responses and relapses was p...
5 CitationsSource
#1Andrew BlauveltH-Index: 54
#2M. S. de Bruin-Weller (UU: Utrecht University)H-Index: 27
Last. B. Shumel (Regeneron)H-Index: 7
view all 31 authors...
Summary Background Dupilumab (an anti-interleukin-4-receptor-α monoclonal antibody) blocks signalling of interleukin 4 and interleukin 13, type 2/Th2 cytokines implicated in numerous allergic diseases ranging from asthma to atopic dermatitis. Previous 16-week monotherapy studies showed that dupilumab substantially improved signs and symptoms of moderate-to-severe atopic dermatitis with acceptable safety, validating the crucial role of interleukin 4 and interleukin 13 in atopic dermatitis pathoge...
179 CitationsSource
#1Namita A. Gandhi (Regeneron)H-Index: 4
#2Gianluca PirozziH-Index: 17
Last. Neil M.H. Graham (Regeneron)H-Index: 18
view all 3 authors...
ABSTRACTIntroduction: Allergy results from an aberrant Type 2 inflammatory response, triggered by a wide range of environmental antigens (allergens) that lead to various immune responses, culminating in the production of immunoglobulin E (IgE). Two key cytokines, interleukin (IL)-4 and IL-13, are critical to the induction and perpetuation of the Type 2 response, and have been implicated in multiple atopic diseases.Area covered: This review summarizes recent milestone developments that have eluci...
32 CitationsSource
#1Terumi KamisawaH-Index: 56
#2Yoh Zen (Kobe University)H-Index: 63
Last. John H. Stone (Harvard University)H-Index: 70
view all 4 authors...
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated condition that can affect almost any organ and is now being recognized with increasing frequency. IgG4-RD is characterized by a lymphoplasmacytic infiltrate composed of IgG4+ plasma cells, storiform fibrosis, obliterative phlebitis, and mild to moderate eosinophilia. The diagnosis of IgG4-RD unifies many eponymous fibroinflammatory conditions that had previously been thought to be confined to single organs. IgG4-RD lesions ...
368 CitationsSource
#1Blanka M. Kaplan (Hofstra University)H-Index: 2
#2Yelena Kopyltsova (North Shore-LIJ Health System)H-Index: 2
Last. Vincent R. BonaguraH-Index: 36
view all 5 authors...
Background As the indications and use of rituximab continue to expand, the reports of long-term effects of anti-CD20–mediated B-cell depletion on the immune system accumulate. Objective We report a group of patients with immunodeficiency who were treated with rituximab and present their immunologic data. Methods A retrospective chart review identified patients with immunodeficiency who received rituximab for treatment of their primary disease and required immunoglobulin replacement therapy (IGRT...
48 CitationsSource
#1Hisanori Umehara (Kanazawa Medical University)H-Index: 24
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 63
Last. Hirotaka Ohhara (Nagoya City University)H-Index: 39
view all 26 authors...
Background IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4+ plasma cells. Although IgG4-RD is not rare and is clinically important, its clinical diagnostic criteria have not been established. Comprehensive diagnostic criteria for IgG4-RD, including the involvement of various organs, are intended for the practical use of general physicians and nonspecialists.
812 CitationsSource
Cited By7
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We thank Ebbo et al for their interest in our case report1 and for providing their case of a patient with IgG4-related disease (IgG4-RD) whom they have also treated with dupilumab.2 Notably, Ebbo et al reported variability in the response to dupilumab compared with our patient. Our patient did have different clinical manifestations than that of Ebbo et al ’s patient, as our patient had profound retroperitoneal and genitourinary fibrosis along with atopic dermatitis, asthma, allergic rhinoconjunc...
Source
#1Motohisa Yamamoto (UTokyo: University of Tokyo)
#2Noritada Yoshikawa (UTokyo: University of Tokyo)H-Index: 18
Last. Hirotoshi Tanaka (UTokyo: University of Tokyo)H-Index: 43
view all 3 authors...
We read with great interest the article from Simpson et al on the clinical efficacy of dupilumab in a patient with IgG4-related disease (IgG4-RD).1 Glucocorticoid is currently the first-line induction therapy for IgG4-RD.2 Since it generally suppresses acquired immune cells, we could not know the therapeutic targets in IgG4-RD. So far, the efficacy of rituximab, targeting B cells, has been discussed,3 but we reconfirm that type 2 helper T (Th2) cells can be one of the therapeutic targets in IgG4...
Source
We thank Della-Torre et al for their interest in our paper and for providing their thoughts through correspondence.1 2 As they have summarised, our case described a patient whom was prescribed a 40 mg daily dose of prednisone to treat his IgG4-related disease (IgG4-RD); however, the patient declined to pursue additional adjunct immunosuppressants due to the concern of adverse effects.1 To hopefully mitigate such concerns and control his disease, we proposed that dupilumab, a monoclonal anti-inte...
1 CitationsSource
We thank Akiyama et al , for their kind words regarding our manuscript.1 We agree that IgG4-related disease (IgG4-RD) relapse frequently occurs in patients tapering glucocorticoids, and we wanted to offer a novel solution to our patient to potentially mitigate or all together avoid this. As communicated in our previous correspondence response2 it has actually come to our attention post-publication (December 29th, 2019) that the patient noted in our report never took a dose of prednisone that was...
Source
#1Mikael Ebbo (AMU: Aix-Marseille University)H-Index: 18
Last. Nicolas Schleinitz (AMU: Aix-Marseille University)H-Index: 28
view all 7 authors...
We read with interest the letter by Simpson et al reporting the use of dupilumab in a patient with IgG4-related disease (IgG4-RD).1 Indeed blocking the IL-4/IL-13 pathway is tempting, considering the roles of these cytokines and the physiopathological changes observed during IgG4-RD.2 3 We have also treated with dupilumab a 51-year-old man with IgG4-related dacryoadenitis and sialadenitis (bilateral lacrimal, parotid, sublingual and submandibular involvement, pattern of Mikulicz syndrome). Left ...
1 CitationsSource
#1Mitsuhiro Akiyama (Keio: Keio University)H-Index: 7
#2Yuko Kaneko (Keio: Keio University)H-Index: 17
Last. Tsutomu TakeuchiTsukasa (Keio: Keio University)H-Index: 63
view all 3 authors...
We read the manuscript reported by Simpson et al with great interest.1 They showed remarkable effectiveness of dupilumab, a monoclonal antibody targeting the interleukin-4 (IL-4) receptor alpha, in immunoglobulin G4-related disease (IgG4-RD) complicated with retroperitoneal fibrosis for the first time. Considering the frequent relapse in patients with IgG4-RD during glucocorticoid tapering and the difficulty in glucocorticoid withdrawal,2 it is of great value that their patient could discontinue...
1 CitationsSource
#1Emanuel Della-Torre (UniSR: Vita-Salute San Raffaele University)H-Index: 14
#2Marco Lanzillotta (UniSR: Vita-Salute San Raffaele University)H-Index: 6
Last. Mona-Rita Yacoub (UniSR: Vita-Salute San Raffaele University)H-Index: 6
view all 3 authors...
We read with interest the article from Simpson et al on the efficacy of dupilumab—an anti-IL-4 receptor alfa monoclonal antibody—in a patient with multi-organ IgG4-related disease (IgG4-RD) involving the retroperitoneum and, apparently, the prostate and the parotid glands.1 According to the case presentation, the patient refused immunosuppressive agents due to the risk of adverse events, and treating physicians decided to start him on 40 mg oral prednisone. Subcutaneous dupilumab was added based...
1 CitationsSource