PT  - JOURNAL ARTICLE
AU  - Masaki, Y
AU  - Dong, L
AU  - Kurose, N
AU  - Kitagawa, K
AU  - Morikawa, Y
AU  - Yamamoto, M
AU  - Takahashi, H
AU  - Shinomura, Y
AU  - Imai, K
AU  - Saeki, T
AU  - Azumi, A
AU  - Nakada, S
AU  - Sugiyama, E
AU  - Matsui, S
AU  - Origuchi, T
AU  - Nishiyama, S
AU  - Nishimori, I
AU  - Nojima, T
AU  - Yamada, K
AU  - Kawano, M
AU  - Zen, Y
AU  - Kaneko, M
AU  - Miyazaki, K
AU  - Tsubota, K
AU  - Eguchi, K
AU  - Tomoda, K
AU  - Sawaki, T
AU  - Kawanami, T
AU  - Tanaka, M
AU  - Fukushima, T
AU  - Sugai, S
AU  - Umehara, H
TI  - 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
AID  - 10.1136/ard.2008.089169
DP  - 2009 Aug 01
TA  - Annals of the Rheumatic Diseases
PG  - 1310--1315
VI  - 68
IP  - 8
4099  - http://ard.bmj.com/content/68/8/1310.short
4100  - http://ard.bmj.com/content/68/8/1310.full
SO  - Ann Rheum Dis2009 Aug 01; 68
AB  - 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 (>135 mg/dl) and infiltration of IgG4+ plasma cells in the tissue (IgG4+/IgG+ plasma cells >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.