Portal de Periódicos da CAPES

Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders

2008; BMJ; Volume: 68; Issue: 8 Linguagem: Inglês

10.1136/ard.2008.089169

1468-2060

Y Masaki, Lei Dong, Nobuyuki Kurose, K. Kitagawa, Yukie Morikawa, Miho Yamamoto, Hidenobu Takahashi, Yasuhisa Shinomura, Koji Imai, Takako Saeki, Atsushi Azumi, Shinji Nakada, Eiji Sugiyama, Shoko Matsui, Tomoki Origuchi, Susumu Nishiyama, Isao Nishimori, Takayuki Nojima, Ken‐ichi Yamada, Motoko Kawano, Yoh Zen, Masanori Kaneko, Kana Miyazaki, Kazuo Tsubota, Kana Eguchi, Koichi Tomoda, Toshioki Sawaki, Takafumi Kawanami, Makoto Tanaka, Toshihiro Fukushima, S Sugai, Hisanori Umehara,

Neuroendocrine Tumor Research Advances

Background: Mikulicz's disease (MD) has been considered as one manifestation of Sjögren'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.