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

Yasukawa, Masaki; Dong, Lingli; Kurose, Nozomu; Kitagawa, Kazuko; Morikawa, Yuko; Yamamoto, Motohisa; Takahashi, Hiroki; Shinomura, Yasuhisa; Imai, Kohzoh; Saeki, Takako; Azumi, Atsushi; Nakada, Shinji; Sugiyama, Eiji; Matsui, Shoko; Origuchi, Tomoki; Nishiyama, Susumu; Nishimori, Isao; Nojima, Takayuki; Yamada, Kazunori; Kawano, Mitsuhiro; Zen, Yoh; Kaneko, Masayuki; Miyazaki, Kana; Tsubota, Kazuo; Eguchi, Katsumi; Tomoda, Kouichi; Sawaki, Toshioki; Kawanami, Takafumi; Tanaka, Masao; Fukushima, Toshihiro; Sugai, Susumu; Umehara, Hisanori

doi:10.1136/ard.2008.089169

Cited by 539 publications

(466 citation statements)

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“…We diagnosed IgG4-RD according to the provisional diagnostic criteria for IgG4-RD of Masaki et al [25]. Renal biopsies were performed in six patients in whom IgG4-related TIN was highly suspected because of renal dysfunction, elevation of renal tubular markers, or abnormal imaging finding, and we diagnosed them with IgG4-related TIN based on diagnostic criteria proposed by Kawano et al [26] and Raissian et al [22].…”

Section: Methodsmentioning

confidence: 99%

Clinical and histological changes associated with corticosteroid therapy in IgG4-related tubulointerstitial nephritis

et al. 2012

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ObjectivesThis study aimed to investigate the clinicopathological changes induced by corticosteroid therapy in immunoglobulin (Ig)G4-related tubulointerstitial nephritis (TIN).MethodsWe studied six IgG4-related TIN patients receiving renal biopsies before and after corticosteroid therapy. Their clinical data and histological findings were evaluated before and after therapy.ResultsElevated serum creatinine levels rapidly improved after corticosteroid therapy except for two patients, in whom it persisted. Abnormal radiological findings improved in all patients, although focal cortical atrophy persisted in three. Histologically, TIN-like dense lymphoplasmacytic infiltration, interstitial fibrosis, IgG4-positive plasma cell, CD4+CD25+ T cell, and Foxp3+ cell infiltration were characteristic before therapy. After therapy, the area with cell infiltration decreased and regional fibrosis became evident in the renal interstitium. The number of IgG4-positive plasma cells and Foxp3+ cells significantly diminished even in the early stage of therapy, whereas low to moderate numbers of CD4+ and CD8+ T cells still infiltrated where inflammation persisted in the later stage.ConclusionsOur study shows that persistent renal insufficiency associated with macroscopic atrophy and microscopic fibrosis is not so rare in IgG4-related TIN. Pathologically, the behavior of regulatory T cells during the clinical course is quite similar to that of IgG4-positive plasma cells, and the behavior pattern of those cells is distinctive.

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Section: Methodsmentioning

confidence: 99%

Clinical and histological changes associated with corticosteroid therapy in IgG4-related tubulointerstitial nephritis

et al. 2012

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“…4b); (3) serum IgG4 and IgE concentrations were significantly higher in IgG4RD than in SS patients (Fig. 4c); and (4) steroid therapy was extremely effective in patients with IgG4RD but had limited effect in patients with SS [29]. …”

Section: Clinicopathological Features Of Igg4rdmentioning

confidence: 99%

“…Comparison of clinical symptoms and laboratory findings in IgG4RD and typical Sjögren’s syndrome ( SS ) [29]. a Clinical symptoms, b immunological findings, and c subclasses of immunoglobulins and IgG observed in patients with IgG4RD ( n = 61) and typical SS ( n = 31).…”

Section: Clinicopathological Features Of Igg4rdmentioning

confidence: 99%

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A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details

et al. 2011

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IgG4-related disease (IgG4RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4RD may be present in a certain proportion of patients with a wide variety of diseases, including Mikulicz’s disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudotumor. Although IgG4RD forms a distinct, clinically independent disease category and is attracting strong attention as a new clinical entity, many questions and problems still remain to be elucidated, including its pathogenesis, the establishment of diagnostic criteria, and the role of IgG4. Here we describe the concept of IgG4RD and up-to-date information on this emerging disease entity.

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“…Such abnormalities include an elevated serum IgG4 level and numerous IgG4-positive plasma cells infiltrating the affected tissue, and these disorders are classified as IgG4-related diseases. [1][2][3][4][5][6][7][8][9][10][11] Castleman's disease is a rather rare atypical lymphoproliferative disorder 12 classified according to the histopathologic findings of the affected lymph nodes as plasma cell type, hyaline-vascular type or a mixed-type variant of the two. 13,14 Patients with the plasma cell or the mixed-type variant frequently have systemic manifestations (so-called multicentric Castleman's disease), such as low-grade fever, fatigue, loss of appetite, and weight loss.…”

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confidence: 99%

Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman's disease

Kojima²,

et al. 2009

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

Abstract: 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.

Cited by 539 publications

References 23 publications

Clinical and histological changes associated with corticosteroid therapy in IgG4-related tubulointerstitial nephritis

Clinical and histological changes associated with corticosteroid therapy in IgG4-related tubulointerstitial nephritis

A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details

Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman's disease

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