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.

Abstract

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.

• Authors: Tomoko Miyashita, Katsunobu Yoshioka, Tomoyuki Nakamura, Yuki Kubo, Takeshi Inoue, Takashi Morikawa, Keiichi Ishii, Keiko Yamagami
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 49 Issue 18 Pg. 2007-11 ( 2010) ISSN: 1349-7235 [Electronic] Japan
• PMID: 20847508 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Immunoglobulin G
• Prednisolone

Topics

• Chemotaxis, Leukocyte (drug effects)
• Humans
• Immunoglobulin G (biosynthesis, blood)
• Lung Neoplasms (blood, chemically induced, pathology)
• Lymphomatoid Granulomatosis (blood, chemically induced, pathology)
• Male
• Middle Aged
• Plasma Cells (drug effects, pathology)
• Prednisolone (adverse effects, therapeutic use)