The immunobiology and clinical characteristics of IgG4 related diseases

Journal of Autoimmunity
H TakahashiKohzoh Imai

Abstract

Having the characteristic features of elevated serum IgG4 levels and prominent infiltration of IgG4-positive plasma cells with fibrosis in lesions, Mikulicz's disease (MD) has been recognized as an IgG4-related disease (IgG4-RD). Although incidence of autoimmune pancreatitis (AIP), one of the organ characteristics of IgG4-RD, has been internationally reported, there are only a few such reports of IgG4-related MD. The limited number of reports might be attributable to the low recognition of IgG4-related MD as a clinical entity as well as its misdiagnosis as Sjögren's syndrome (SS). Thus, we compared several clinical features of MD with SS to improve proper clinical diagnosis of MD in the clinical setting. A total of 70 SS and 70 MD cases evaluated at Sapporo Medical University Hospital were retrospectively analyzed. In SS patients, sicca symptoms were the most frequent (87%), followed by articular symptoms (23%), while lacrimal and salivary gland swelling were a rare (10%) and transient manifestation. In contrast, lacrimal or salivary gland swelling was observed in all patients with MD. Although nearly 60% of MD patients complained of sicca syndrome, skin rash and arthralgia were rare symptoms. Hypergammaglobulinemia was recogni...Continue Reading

References

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Citations

Jan 7, 2014·Nihon Rinshō Men'eki Gakkai kaishi = Japanese journal of clinical immunology·Hiroki TakahashiKohzoh Imai
Dec 4, 2013·Nature Reviews. Rheumatology·Motohisa YamamotoYasuhisa Shinomura
Aug 26, 2014·Autoimmunity Reviews·Pilar Brito-ZerónJohn H Stone
Jan 18, 2014·Ocular Immunology and Inflammation·Liyang Wang, Weiran Niu
Mar 19, 2013·Journal of Autoimmunity·M J BarreraM J González
Oct 9, 2012·Best Practice & Research. Clinical Rheumatology·Monica Guma, Gary S Firestein
Apr 4, 2015·Case Reports in Rheumatology·Teresa CarboneLuis Mocarzel
Oct 21, 2015·European Journal of Internal Medicine·Lilian Vasaitis
Mar 5, 2016·Surgical Pathology Clinics·Christine E Bookhout, Marian A Rollins-Raval
Jul 17, 2014·Orphanet Journal of Rare Diseases·Herwig PieringerJochen Zwerina
Jun 16, 2012·International Journal of Rheumatology·Laura C LighaamTheo Rispens
Aug 11, 2015·International Journal of Rheumatic Diseases·Kanae Kubo, Kazuhiko Yamamoto
May 20, 2015·Arthritis & Rheumatology·Zachary S WallaceJohn H Stone
May 23, 2015·The Laryngoscope·Xia HongGuang-Yan Yu
Jul 5, 2015·Mayo Clinic Proceedings·John H StoneManuel Ramos-Casals
Feb 4, 2014·Journal of Autoimmunity·Haralampos M Moutsopoulos
Oct 26, 2013·Autoimmunity Reviews·Karmtej SinghM Eric Gershwin
Aug 16, 2016·Clinics in Chest Medicine·Jay H Ryu, Eunhee S Yi
Jul 10, 2018·BMJ Case Reports·Kai-Chun WangChang-Youh Tsai
Feb 13, 2015·Chinese Medical Journal·Jian-Min MaXiao-Na Wang
May 23, 2015·Journal of Clinical Neuromuscular Disease·Waqar WaheedRup Tandan

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