A case of lymphomatoid granulomatosis-like lung lesions with abundant infiltrating IgG4-positive plasma cells whose serum IgG4 levels became high following the start of corticosteroid therapy.
A 59-year-old man with a history of prostate hyperplasia was admitted to our hospital for further examination of a lung mass and renal dysfunction. Lung biopsy specimens revealed that inflammatory cells had infiltrated into the blood vessel walls. We initially suspected lymphomatoid granulomatosis, but Epstein Barr virus-encoded small RNA was negative. However, 50% of the infiltrating plasma cells were positive for IgG4. Furthermore, the kidneys and prostate contained abundant IgG4-positive plasma cells. He was diagnosed with IgG4-related sclerosing disease even though serum IgG4 levels were not elevated (45.7 mg/dL). Prednisolone reduced the lung masses and ameliorated renal function, but the serum IgG4 level increased (377 mg/dL). Seronegative IgG4-related sclerosing disease should be considered when patients present with such symptoms and treatment responses, and the secretion of IgG4 might be blocked by its active synthesis.
Miyashita
Tomoko
T
Department of Internal Medicine, Osaka City General Hospital, Osaka. minet56@yahoo.co.jp
Yoshioka
Katsunobu
K
Nakamura
Tomoyuki
T
Kubo
Yuki
Y
Inoue
Takeshi
T
Morikawa
Takashi
T
Ishii
Keiichi
K
Yamagami
Keiko
K
D002634:Chemotaxis, Leukocyte / Q000187:drug effects; D006801:Humans; D007074:Immunoglobulin G / Q000096:biosynthesis / Q000097:blood*; D008175:Lung Neoplasms / Q000097:blood / Q000139:chemically induced* / Q000473:pathology; D008230:Lymphomatoid Granulomatosis / Q000097:blood / Q000139:chemically induced* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000187:drug effects / Q000473:pathology*; D011239:Prednisolone / Q000009:adverse effects* / Q000627:therapeutic use
D007074:Immunoglobulin G; D011239:Prednisolone