Abstract / Summary
Abstract Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disorder characterized by tissue infiltration with IgG4-positive plasma cells and storiform fibrosis. Spinal hypertrophic pachymeningitis (SHP) is a rare manifestation of this disease, characterized by dural thickening, which can result in significant spinal cord compression. This report discusses the case of a 50-year-old female who presented with subacute quadriparesis, and was eventually diagnosed with IgG4-related SHP. This case illustrates that early surgical decompression is a highly effective treatment for IgG4-related SHP, can facilitate rapid and sustainable recovery. Also, this is the first reported case that demonstrates anti-nRNP antibodies positivity in a patient with IgG4-related SHP.