Analysis of Serum IgG Subclasses in Churg-Strauss Syndrome -The Meaning of Elevated Serum Levels of IgG4

Motohisa Yamamoto; Hiroki Takahashi; Chisako Suzuki; Tetsuya Tabeya; Mikiko Ohara; Yasuyoshi Naishiro; Hiroyuki Yamamoto; Kohzoh Imai; Yasuhisa Shinomura

doi:10.2169/internalmedicine.49.3532

ORIGINAL ARTICLES

Analysis of Serum IgG Subclasses in Churg-Strauss Syndrome -The Meaning of Elevated Serum Levels of IgG4

Motohisa Yamamoto, Hiroki Takahashi, Chisako Suzuki, Tetsuya Tabeya, Mikiko Ohara, Yasuyoshi Naishiro, Hiroyuki Yamamoto, Kohzoh Imai, Yasuhisa Shinomura

Author information

Motohisa Yamamoto
The First Department of Internal Medicine, Sapporo Medical University School of Medicine
Hiroki Takahashi
The First Department of Internal Medicine, Sapporo Medical University School of Medicine
Chisako Suzuki
The First Department of Internal Medicine, Sapporo Medical University School of Medicine
Tetsuya Tabeya
The First Department of Internal Medicine, Sapporo Medical University School of Medicine
Mikiko Ohara
The First Department of Internal Medicine, Sapporo Medical University School of Medicine
Yasuyoshi Naishiro
The First Department of Internal Medicine, Sapporo Medical University School of Medicine
Hiroyuki Yamamoto
The First Department of Internal Medicine, Sapporo Medical University School of Medicine
Kohzoh Imai
The First Department of Internal Medicine, Sapporo Medical University School of Medicine
Yasuhisa Shinomura
The First Department of Internal Medicine, Sapporo Medical University School of Medicine

Keywords: allergy, autoimmune pancreatitis, Churg-Strauss syndrome, IgG4, Mikulicz's disease, IgG4-related disease

JOURNAL OPEN ACCESS

2010 Volume 49 Issue 14 Pages 1365-1370

DOI https://doi.org/10.2169/internalmedicine.49.3532

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Abstract

Objective Mikulicz's disease (MD) is characterized by symmetrical and persistent enlargement of the lacrimal and salivary glands. Recently it has been categorized as an 'Ig (immunoglobulin) G4-related disease.' It presents with elevated serum levels of IgG4 and abundant infiltration of IgG4-bearing plasmacytes in involved organs. Allergic symptoms are often observed in patients with IgG4-related disease. On the other hand, allergic diseases are often complicated with Churg-Strauss syndrome (CSS). Here we focused on CSS and analyzed the relation of IgG4 in its pathogenesis.
Materials and Methods We analyzed five patients (2 men and 3 women) with CSS and 51 patients (20 men and 31 women) with MD who presented at Sapporo Medical University Hospital since 2001. We measured the serum concentrations of IgG subclasses in the patients with MD and CSS, and evaluated renal specimens from CSS patients, staining them for anti-IgG4 antibody.
Results We surprisingly found elevated serum levels of IgG4 not only in MD but also in CSS patients. The renal specimens in CSS patients revealed the infiltration of IgG4-positive plasmacytes.
Conclusion IgG4-bearing plasmacytes may be involved in the pathogenesis of CSS, and it is possible that an allergic reaction plays an important role in the pathogenesis of IgG4-related disease.

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