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

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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.<br>


  • Internal Medicine

    Internal Medicine 49(18), 2007-2011, 2010

    The Japanese Society of Internal Medicine


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