Immunoglobulin G4-related hypertrophic pachymeningitis with spinal cord compression: A case report

• Published in: Journal of neuroimmunology Vol. 347; p. 577325
• Main Authors: Li, Hong-xing, Zuo, Lin, Peng, Xiao-xiao, Zong, Qiang, Zhang, Kai, Liu, Yi-zhe, Wang, Han, Han, Guang-liang
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 15.10.2020
• Subjects: Female; Humans; Hypertrophic pachymeningitis; IgG4-related disease (IgG4-RD); Immunoglobulin G4-Related Disease - complications; Immunoglobulin G4-Related Disease - diagnostic imaging; Immunoglobulin G4-Related Disease - surgery; Meningitis - complications; Meningitis - diagnostic imaging; Meningitis - surgery; Middle Aged; Spinal cord compression; Spinal Cord Compression - complications; Spinal Cord Compression - diagnostic imaging; Spinal Cord Compression - surgery; Thoracic Vertebrae - diagnostic imaging; Thoracic Vertebrae - surgery; IgG4-HP; IgG4-related disease (IgG4-RD); MRI; Hypertrophic pachymeningitis; Spinal cord compression; IgG4; CNS; IgG4-RD; HPF
• ISSN: 0165-5728; 1872-8421
• DOI: 10.1016/j.jneuroim.2020.577325

IgG4-related disease (IgG4-RD) is a recently recognized inflammatory condition that can be found in many organs. However, spinal involvement is rare and has been described only in case reports and series. Here, we report a rare case of spinal IgG4-RD that resulted in hypertrophic pachymeningitis with spinal cord compression. This case expands the phenotypic presentation for the neurological sequelae of IgG4-RD. Our case hints that spinal IgG4-RD may be misdiagnosed, and IgG4-RD in patients should be considered when the patient has a dural mass. Although early surgery, steroids, and/or immunosuppressive therapy may prevent neurological complications, the side effects should receive more attention during treatment. [Display omitted] •Spinal IgG4-related disease is rare, which may be misdiagnosed in clinical work.•Spinal IgG4-related disease in patients should be considered when the patient with dural mass and spinal cord compression.•Pathological examination is the key to diagnose IgG4- related disease.