Immunoglobulin G4-related spinal pachymeningitis

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is rare disease entity and has recently been recognized as an inflammatory disorder with the tendency to affect multiple organs. Pachymeningitis of spine caused by IgG4-related disease is extremely rare. Neck pain and spinal cord compression symptoms consist of usual presentation of IgG4-related spinal pachymeningitis; however, polyarthritis is an unusual presentation of this disease, and it was reported in only one case that mimicked psoriatic arthritis. In this report, we describe a case of IgG4-related spinal pachymeningitis in a middle-age male who presented initially with neck pain and rheumatoid arthritis-like symptoms and later on developed both right upper and lower limb weakness. He was found to had pachymeningeal thickening on a cervical magnetic resonance image (MRI) and elevated serum IgG4 levels. He was treated with glucocorticoids and rituximab, which led to a significantly positive radiological response.

Figure 1

A) Sagital and B) axial T1-Fat Sat postcontrast MR showing thickening extending from the dorsal aspect of clivus to T1 level with cord compression (arrow). The non-enhancing and low signal anterior to the enhancing component is consistent with chronic fibrotic changes (arrow head).

Figure 2

A) Sagittal and B) axial showing signi cant improvement after 4 months of therapy with corticosteroids and rituximab treatment (arrow). e degree of enhancement has improved; however, the non enhancing component remains unchanged(arrow head).