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.