Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders
Yasufumi Masaki,Lingli Dong,Lingli Dong,Nozomu Kurose,Kazuko Kitagawa,Yuko Morikawa,Motohisa Yamamoto,Hiroki Takahashi,Yasuhisa Shinomura,Kohzoh Imai,Takako Saeki,Atsushi Azumi,Shinji Nakada,Eiji Sugiyama,Shoko Matsui,Tomoki Origuchi,Susumu Nishiyama,Isao Nishimori,Takayuki Nojima,Kazunori Yamada,Mitsuhiro Kawano,Yoh Zen,Masahiko Kaneko,Kana Miyazaki,Kazuo Tsubota,Katsumi Eguchi,Kouichi Tomoda,Toshioki Sawaki,Takafumi Kawanami,Masao Tanaka,Toshihiro Fukushima,Susumu Sugai,Hisanori Umehara +32 more
TLDR
There are considerable clinical and pathological differences between IgG4+MOLPS and SS, and based on the clinical features and good response to glucocorticoids, a new clinical entity is proposed: IgG 4+MolPS.Abstract:
Background: Mikulicz’s disease (MD) has been considered as one manifestation of Sjogren’s syndrome (SS). Recently, it has also been considered as an IgG 4 -related disorder. Objective: To determine the differences between IgG 4 -related disorders including MD and SS. Methods: A study was undertaken to investigate patients with MD and IgG 4 -related disorders registered in Japan and to set up provisional criteria for the new clinical entity IgG 4 -positive multiorgan lymphoproliferative syndrome (IgG 4 +MOLPS). The preliminary diagnostic criteria include raised serum levels of IgG 4 (>135 mg/dl) and infiltration of IgG 4 + plasma cells in the tissue (IgG 4 +/IgG+ plasma cells >50%) with fibrosis or sclerosis. The clinical features, laboratory data and pathologies of 64 patients with IgG 4 +MOLPS and 31 patients with typical SS were compared. Results: The incidence of xerostomia, xerophthalmia and arthralgia, rheumatoid factor and antinuclear, antiSS-A/Ro and antiSS-B/La antibodies was significantly lower in patients with IgG 4 +MOLPS than in those with typical SS. Allergic rhinitis and autoimmune pancreatitis were significantly more frequent and total IgG, IgG 2 , IgG 4 and IgE levels were significantly increased in IgG 4 +MOLPS. Histological specimens from patients with IgG 4 +MOLPS revealed marked IgG 4 + plasma cell infiltration. Many patients with IgG 4 +MOLPS had lymphocytic follicle formation, but lymphoepithelial lesions were rare. Few IgG 4 + cells were seen in the tissue of patients with typical SS. Thirty-eight patients with IgG 4 +MOLPS treated with glucocorticoids showed marked clinical improvement. Conclusion: Despite similarities in the involved organs, there are considerable clinical and pathological differences between IgG 4 +MOLPS and SS. Based on the clinical features and good response to glucocorticoids, we propose a new clinical entity: IgG 4 +MOLPS.read more
Citations
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Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011
Hisanori Umehara,Kazuichi Okazaki,Yasufumi Masaki,Mitsuhiro Kawano,Motohisa Yamamoto,Takako Saeki,Shoko Matsui,Tadashi Yoshino,Shigeo Nakamura,Shigeyuki Kawa,Hideaki Hamano,Terumi Kamisawa,Toru Shimosegawa,Akira Shimatsu,Seiji Nakamura,Tetsuhide Ito,Kenji Notohara,Takayuki Sumida,Yoshiya Tanaka,Tsuneyo Mimori,Tsutomu Chiba,Michiaki Mishima,Toshifumi Hibi,Hirohito Tsubouchi,Kazuo Inui,Hirotaka Ohara +25 more
TL;DR: The comprehensive diagnostic criteria for IgG4-RD are practically useful for general physicians and nonspecialists and have increased the sensitivity of diagnosis to 100% for Igg4-related MD, KD, and AIP.
Journal ArticleDOI
American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort
Stephen Shiboski,Caroline H. Shiboski,Lindsey A. Criswell,Alan N. Baer,Stephen Challacombe,Hector Lanfranchi,Morten Schiødt,Hisanori Umehara,Frederick B. Vivino,Yongju Zhao,Yan Dong,Deborah Greenspan,Ana Maria Heidenreich,Pekka Helin,Bruce Kirkham,Kazuko Kitagawa,Genevieve Larkin,Mengtao Li,Thomas M. Lietman,J Lindegaard,Nancy A. McNamara,Kenneth E. Sack,P. J. Shirlaw,Susumu Sugai,C. Vollenweider,John P. Whitcher,Ava J. Wu,S. Zhang,W Zhang,John S. Greenspan,Troy E. Daniels +30 more
TL;DR: New classification criteria for Sjögren's syndrome are proposed, which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity.
Journal ArticleDOI
IgG4-related disease.
Book ChapterDOI
Comprehensive Diagnostic Criteria for IgG4-Related Disease
TL;DR: This work established comprehensive diagnostic criteria for IgG4-RD for the practical use of specialists and nonspecialists and describes the pathological features of each organ differ.
Journal ArticleDOI
IgG4-related disease.
TL;DR: Advances in understanding the pathophysiology of IgG4-RD have proceeded swiftly, leading to the identification of a number of potential targeted treatment strategies, which have direct implications for the development of targeted strategies for the treatment of this condition.
References
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Journal ArticleDOI
Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group
Claudio Vitali,Stefano Bombardieri,Roland Jonsson,Haralampos M. Moutsopoulos,E L Alexander,Steven E. Carsons,Troy E. Daniels,P C Fox,Robert I. Fox,Stuart S. Kassan,S R Pillemer,Norman Talal,M H Weisman +12 more
TL;DR: These classification criteria for Sjögren's syndrome have been re-examined by consensus group members, who have introduced some modifications, more clearly defined the rules for classifying patients with primary or secondary SS, and provided more precise exclusion criteria.
Journal ArticleDOI
High serum IgG4 concentrations in patients with sclerosing pancreatitis.
Hideaki Hamano,Shigeyuki Kawa,Akira Horiuchi,Hiroshi Unno,Naoyuki Furuya,Taiji Akamatsu,Mana Fukushima,Toshio Nikaido,Kohzo Nakayama,Nobuteru Usuda,Kendo Kiyosawa +10 more
TL;DR: Patients with sclerosing pancreatitis have high serum IgG4 concentrations, providing a useful means of distinguishing this disorder from other diseases of the pancreas or biliary tract.
Journal ArticleDOI
A new clinicopathological entity of IgG4-related autoimmune disease
Terumi Kamisawa,Nobuaki Funata,Yukiko K. Hayashi,Yoshinobu Eishi,Morio Koike,Kouji Tsuruta,Atsutake Okamoto,Naoto Egawa,Hitoshi Nakajima +8 more
TL;DR: It is suggested that AIP is not simply pancreatitis but that it is a pancreatic lesion involved in IgG4-related systemic disease with extensive organ involvement, and a new clinicopathological entity is proposed.
Journal ArticleDOI
IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis : Do they belong to a spectrum of sclerosing pancreatitis?
Yoh Zen,Kenichi Harada,Motoko Sasaki,Yasunori Sato,Koichi Tsuneyama,Joji Haratake,Hiroshi Kurumaya,Kazuyoshi Katayanagi,Shinji Masuda,Hideki Niwa,Hideo Morimoto,Atsuo Miwa,Akio Uchiyama,Bernard Portmann,Yasuni Nakanuma +14 more
TL;DR: 4 SC cases and all SC-hepatic IP cases showed bile duct lesions identical to those of SP-SC, suggesting that these three conditions may be a single disease entity.
Journal Article
A Clinicopathologic Study of “Mikulicz's Disease”
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