Possible relationship between fibrosis of IgG4-related thymitis and the profibrotic cytokines, transforming growth factor beta 1, interleukin 1 beta and interferon gamma: a case report

• DOI: 10.1186/s13000-018-0684-1
• Publication Date: 2018-01-17T12:21:01Z
• Authors: Atsuko Masunaga; Fumihiro Ishibashi; Eitetsu Koh; Takashi Oide; Yasuo Sekine; Kenzo Hiroshima
• Keywords: IgG4-Related Disease; Infiltration (HVAC)

IgG4-related disease often forms a mass and the affected lesion is clinically removed because cannot be differentiated from neoplasm. Affected lesions commonly occur in pancreas, hepatobiliary tract, kidney, retroperitoneum. However, rarely occurs thymus. A histological worldwide consensus of proposed that pathological diagnosis should meet more than two three major features: 1) dense lymphoplasmacytic infiltration with greater 40% IgG4+/IgG+ plasma cells, 2) storiform fibrosis; 3) obliterative phlebitis. Currently, fibrosis thought to induced by profibrotic cytokines such as transforming growth factor beta 1 (TGFB1), interleukin (IL1B) interferon gamma (IFNG), which are secreted regulatory T cells (Tregs) CD4-positive cytotoxic cells. it unclear whether associated seen thymitis. Here we examined were increased compared those surrounding thymus, Tregs present mass, using reverse transcription absolute quantitative polymerase chain reaction (RT-ab-qPCR) immunohistochemistry. 70-year-old Japanese man contracted IgG4-letated Histological immunohistochemical analyses demonstrated his had massive focally pattern but not The was surrounded atrophic We diagnosed Immunohistochemically, scattered throughout mass. RT-ab-qPCR showed messenger RNA expressions TGFB1, IL1B IFNG 270-, 158- 5.5- fold higher His serum IgG4 level after surgery within normal range (83.4 mg/dl soon surgery, 89.3 2 weeks surgery). Our results suggested induce might produce some these thymitis well other disease.