IgG4-related disease (IgG4-RD) is a systemic lymphoproliferative disease characterized by elevated serum IgG4 values and increased numbers of IgG4-producing plasmacytic cells in tissues. Lesions may develop at sites throughout the body. The disease tends to target some organs frequently, e.g., the pancreas, lacrimal glands, salivary glands, kidneys, and retroperitoneum. Other organs, such as the central nervous system and prostate gland, are affected less frequently. In this chapter, we discuss the central nervous system lesions of hypertrophic pachymeningitis, hypophysitis, and intracerebral inflammatory pseudotumor, as well as the prostate lesions associated with IgG4-RD.
Giant Cell Arteritis Posterior Lobe Pituitary Stalk Central Nervous System Lesion Central Diabetes Insipidus
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