Igg4 Related Granulomatous Spinal Pachymeningitis with Cord Compression – A Multisystem Disorder Presenting as Tumor Mimic

Abstract

IgG4 Related Disease (IgG4 RD) is a comparatively new multisystem disorder associated with elevated levels of IgG4. It includes a number of disorders which were previously thought to be idiopathic or autoimmune. This is a rare condition and can affect any organ system in the body with IgG4 Related pachymeningitis having even lesser incidence. In this case report we present a patient who had chronic low back ache, who on evaluation was found to have a mass compressing on the spinal cord at the thoracic level, requiring surgical decompression. Biopsy and immunological testing were suggestive of IgG4 related pachymeningitis. We describe the diagnosis, medical management, and follow up of this patient, with discussion on how timely diagnosis and management will help in preventing further episodes and help in recovery. This case reiterates about medical conditions presenting as tumor mimics.