IgG4-associated sclerosing pachymeningitis
Monday 15 November 2010
IgG4-related sclerosing disease is a distinctive mass-forming lesion with frequent systemic involvement, most frequently the pancreas, salivary glands, and lacrimal glands.
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.
central nervous system involvement
spinal cord compression
elongated dural mass
dense lymphoplasmacytic infiltrate
IgG4+ plasma cells increased
proportion of IgG4+/IgG+ plasma cells = 85%
bilateral submandibular swelling (chronic sialadenitis)
IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Chan SK, Cheuk W, Chan KT, Chan JK. Am J Surg Pathol. 2009 Aug;33(8):1249-52. PMID: 19561447