The American journal of surgical pathology
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Am. J. Surg. Pathol. · Aug 2009
Case ReportsIgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease.
IgG4-related sclerosing disease is a distinctive mass-forming lesion with frequent systemic involvement, most frequently the pancreas, salivary glands, and lacrimal glands. This report describes a case manifesting with a previously unrecognized form of central nervous system involvement. The 37-year-old man presented with signs and symptoms of spinal cord compression at the thoracic level 9. ⋯ The patient also had a 1-year history of bilateral submandibular swelling due to chronic sialadenitis. Thus, IgG4-related sclerosing pachymeningitis represents a new member of the IgG4-related sclerosing disease family affecting the central nervous system. It seems that at least a proportion of cases described in the literature as idiopathic hypertrophic pachymeningitis belong to this disease, especially as some patients have other clinical manifestations compatible with IgG4-related sclerosing disease, such as cholangitis and orbital pseudotumor.