IgG4-positive multi-organ lymphoproliferative syndrome manifesting as chronic symmetrical sclerosing dacryo-sialadenitis with subsequent secondary portal hypertension and remarkable IgG4-linked IL-4 elevation

Suzuki, Katsuya; Tamaru, Jun Ichi; Okuyama, Atsushi; Kameda, Hideto; Amano, Koichi; Nagasawa, Hayato; Nishi, Eiko; Yoshimoto, Keiko; Setoyama, Yumiko; Kaneko, Keiko; Osada, Hiroyuki; Honda, N.; Sasaki, Yasutsuna; Itoyama, Shinji; Tsuzaka, Kensei; Takeuchi, Tsutomu

doi:10.1093/rheumatology/keq113

Cited by 33 publications

(21 citation statements)

References 10 publications

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“…Although corticosteroids are generally effective in treating the extravascular lesions of IgG4RD (4,23), the present case did not respond to immunosuppressive therapy including these drugs. Further, given that corticosteroids might increase the risk of aneurysmal rupture by making the vascular wall thinner and more fragile, corticosteroid dose was tapered off in this patient.…”

Section: Discussioncontrasting

confidence: 65%

A Case of Multiple Giant Coronary Aneurysms and Abdominal Aortic Aneurysm Coexisting with IgG4-related Disease

et al. 2012

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IgG4-related disease (IgG4RD) is a unique systemic lymphoproliferative disorder characterized by elevated serum IgG4 levels and IgG4-producing plasma cell expansion in the affected tissues, which are accompanied by fibrotic or sclerotic changes. Vascular lesions may also be a part of IgG4RD as a number of case reports have discussed inflammatory abdominal aortic aneurysms associated with IgG4RD, but coronary artery lesions seem to be rare complications of IgG4RD. A 71-year-old man suffered from multiple giant coronary aneurysms and an abdominal aortic aneurysm with concurrent pancreatic, gall bladder, bile duct, and salivary gland lesions resulting from IgG4RD. The present observations suggest that coronary aneurysms may also develop as a consequence of this disease.

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Section: Discussioncontrasting

confidence: 65%

A Case of Multiple Giant Coronary Aneurysms and Abdominal Aortic Aneurysm Coexisting with IgG4-related Disease

et al. 2012

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“…For example, the Th2-dominant immune response and the production of Th2-type cytokines, such as IL-4, IL-5, IL-10, and IL-13, are increased [69–71]. Furthermore, the numbers of regulatory T cells (Treg) expressing CD4+CD25+Foxp3 are significantly higher in the affected tissues and peripheral blood of patients with IgG4RD than the numbers in patients with autoimmune and nonautoimmune diseases [72–74].…”

Section: Pathogenesis and Pathophysiology Of Igg4rdmentioning

confidence: 99%

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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“…5 Recently, AIP has been reported to be associated with various extrapancreatic lesions in organs such as lacrimal and salivary gland lesions, 6,7 hilar lymphadenopathy, 7,8 interstitial pneumonia, 7,9,10 sclerosing cholangitis, 7,11-14 renal lesions, 7,15,16 retroperitoneal fi brosis, 7,[17][18][19] and prostatic lesions. 7,[20][21][22] These pancreatic lesions and extrapancreatic lesions have been reported as IgG4-related sclerosing disease, 23 systemic IgG4-related plasmatic syndrome (SIPS), 24 and IgG4-positive multiorgan lymphoproliferative syndrome 25 because pathological fi ndings similar to those of AIP (i.e., abundant IgG4-positive plasma cells, lymphoplasmacytic infi ltration, fi brosis) were found. To the best of our knowledge, lesions of branches of the trigeminal nerve associated with AIP or infi ltrated by IgG4-positive plasma cells have not been reported in the Englishlanguage literature.…”

Section: Introductionmentioning

confidence: 99%

Infraorbital nerve swelling associated with autoimmune pancreatitis

Watanabe¹,

Fujinaga²,

Kawakami³

et al. 2011

Jpn J Radiol

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IgG4-positive multi-organ lymphoproliferative syndrome manifesting as chronic symmetrical sclerosing dacryo-sialadenitis with subsequent secondary portal hypertension and remarkable IgG4-linked IL-4 elevation

Cited by 33 publications

References 10 publications

A Case of Multiple Giant Coronary Aneurysms and Abdominal Aortic Aneurysm Coexisting with IgG4-related Disease

A Case of Multiple Giant Coronary Aneurysms and Abdominal Aortic Aneurysm Coexisting with IgG4-related Disease

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

Infraorbital nerve swelling associated with autoimmune pancreatitis

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