TY - JOUR T1 - Proposal for a new clinical entity, IgG<sub>4</sub>-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG<sub>4</sub>-related disorders JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - 1310 LP - 1315 DO - 10.1136/ard.2008.089169 VL - 68 IS - 8 AU - Y Masaki AU - L Dong AU - N Kurose AU - K Kitagawa AU - Y Morikawa AU - M Yamamoto AU - H Takahashi AU - Y Shinomura AU - K Imai AU - T Saeki AU - A Azumi AU - S Nakada AU - E Sugiyama AU - S Matsui AU - T Origuchi AU - S Nishiyama AU - I Nishimori AU - T Nojima AU - K Yamada AU - M Kawano AU - Y Zen AU - M Kaneko AU - K Miyazaki AU - K Tsubota AU - K Eguchi AU - K Tomoda AU - T Sawaki AU - T Kawanami AU - M Tanaka AU - T Fukushima AU - S Sugai AU - H Umehara Y1 - 2009/08/01 UR - http://ard.bmj.com/content/68/8/1310.abstract N2 - Background: Mikulicz’s disease (MD) has been considered as one manifestation of Sjögren’s syndrome (SS). Recently, it has also been considered as an IgG4-related disorder.Objective: To determine the differences between IgG4-related disorders including MD and SS.Methods: A study was undertaken to investigate patients with MD and IgG4-related disorders registered in Japan and to set up provisional criteria for the new clinical entity IgG4-positive multiorgan lymphoproliferative syndrome (IgG4+MOLPS). The preliminary diagnostic criteria include raised serum levels of IgG4 (&gt;135 mg/dl) and infiltration of IgG4+ plasma cells in the tissue (IgG4+/IgG+ plasma cells &gt;50%) with fibrosis or sclerosis. The clinical features, laboratory data and pathologies of 64 patients with IgG4+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 IgG4+MOLPS than in those with typical SS. Allergic rhinitis and autoimmune pancreatitis were significantly more frequent and total IgG, IgG2, IgG4 and IgE levels were significantly increased in IgG4+MOLPS. Histological specimens from patients with IgG4+MOLPS revealed marked IgG4+ plasma cell infiltration. Many patients with IgG4+MOLPS had lymphocytic follicle formation, but lymphoepithelial lesions were rare. Few IgG4+ cells were seen in the tissue of patients with typical SS. Thirty-eight patients with IgG4+MOLPS treated with glucocorticoids showed marked clinical improvement.Conclusion: Despite similarities in the involved organs, there are considerable clinical and pathological differences between IgG4+MOLPS and SS. Based on the clinical features and good response to glucocorticoids, we propose a new clinical entity: IgG4+MOLPS. ER -