IgG4-related sclerosing pachymeningitis causing spinal cord compression.

摘要

Immunoglobulin G (IgG)4-related sclerosing disease is an emerging clinicopathologic entity. It is defined as a systemic disease characterized his-topathologically by extensive IgG4-positive plasma cell infiltration of various organs together with T-lymphocyte infiltration. Pancreas is the most frequently affected organ. This disease was included within the category of autoimmune pancreatitis before the concept of IgG4-related sclerosing disease was introduced. There have been several reports of patients with IgG4-related sclerosing disease involving a wide range of extrapancreatic tissues. However, involvement of the CNS is still exceptional. To date, only 1 case of IgG4-related sclerosing pachymeningitis has been reported in the published literature. We describe IgG4-related sclerosing disease manifesting as an inflammatory epidural mass with spinal cord compression.