RT Journal Article
SR Electronic
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
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP 1310
OP 1315
DO 10.1136/ard.2008.089169
VO 68
IS 8
A1 Masaki, Y
A1 Dong, L
A1 Kurose, N
A1 Kitagawa, K
A1 Morikawa, Y
A1 Yamamoto, M
A1 Takahashi, H
A1 Shinomura, Y
A1 Imai, K
A1 Saeki, T
A1 Azumi, A
A1 Nakada, S
A1 Sugiyama, E
A1 Matsui, S
A1 Origuchi, T
A1 Nishiyama, S
A1 Nishimori, I
A1 Nojima, T
A1 Yamada, K
A1 Kawano, M
A1 Zen, Y
A1 Kaneko, M
A1 Miyazaki, K
A1 Tsubota, K
A1 Eguchi, K
A1 Tomoda, K
A1 Sawaki, T
A1 Kawanami, T
A1 Tanaka, M
A1 Fukushima, T
A1 Sugai, S
A1 Umehara, H
YR 2009
UL http://ard.bmj.com/content/68/8/1310.abstract
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 (&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.