Roles of TGF-beta and periostin in fibrosclerosis in patients with IgG4-related diseases.
CONCLUSIONS
These results suggest that transforming growth factor (TGF)-beta and periostin could be useful as novel biomarkers and therapeutic targets in IgG4-related disease.


OBJECTIVE
IgG4-related disease is an uncommon fibrosclerosing and inflammatory mass-forming disease that can be systemic or can affect single organs. To clarify the roles of TGF-beta, periostin, and interleukin (IL)-13 in the pathogenesis of IgG4-related disease, we studied a total of 36 serum and 11 tissue samples from patients with IgG4-related disease.


METHODS
This was a retrospective clinical study. The patient group consisted of six females and seven males (average age 60 years, range 38-74 years). Serum IgG4 levels, the tissue density of IgG4-positive plasmacytes, and the expression of TGF-beta and periostin in the affected tissues were examined immunohistochemically.


RESULTS
Serum IgG4 levels were elevated in all patients (mean 776.6, range 185-2820 mg/dl), and IgG4-positive plasmacytes were observed in the affected salivary glands. Seven patients with prominent infiltration of the involved glands with IgG4-positive plasmacytes had fatal systemic complications, including pancreatitis, after swelling of the salivary glands. Overexpression of TGF-beta and periostin was observed in affected tissues obtained from these patients.
Department of Otolaryngology, Head and Neck Surgery, Yamagata University Faculty of Medicine , Yamagata.
Nobuo
N
Ohta
Kazuya
K
Kurakami
Akihiro
A
Ishida
Takatoshi
T
Furukawa
Yusuke
Y
Suzuki
Masaru
M
Aoyagi
Atsushi
A
Matsubara
Kenji
K
Izuhara
Seiji
S
Kakehata
D000328:Adult; D000368:Aged; D000888:Antibodies, Anti-Idiotypic / Q000097:blood / Q000276:immunology*; D001327:Autoimmune Diseases / Q000097:blood* / Q000276:immunology; D015815:Cell Adhesion Molecules / Q000096:biosynthesis* / Q000097:blood; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000097:blood* / Q000276:immunology; D012185:Retroperitoneal Fibrosis / Q000097:blood / Q000151:congenital* / Q000276:immunology; D012189:Retrospective Studies; D016212:Transforming Growth Factor beta / Q000096:biosynthesis* / Q000097:blood
D000888:Antibodies, Anti-Idiotypic; D015815:Cell Adhesion Molecules; D007074:Immunoglobulin G; C512835:POSTN protein, human; D016212:Transforming Growth Factor beta