IgG4-associated sclerosing pachymeningitis

IgG4-related sclerosing disease is a distinctive mass-forming lesion with frequent systemic involvement, most frequently the pancreas, salivary glands, and lacrimal glands.

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.

Synopsis

 central nervous system involvement
 spinal cord compression
 elongated dural mass
 dense lymphoplasmacytic infiltrate
 stromal fibrosis
 phlebitis
 IgG4+ plasma cells increased
 proportion of IgG4+/IgG+ plasma cells = 85%

Associations

 bilateral submandibular swelling (chronic sialadenitis)

References

 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