LAUSR

BACKGROUND Spinal immunoglobulin G4-related hypertrophic pachymeningitis (IgG4-HP) is a rare disease. Little information is known regarding the diagnosis, management and prognosis of patients with spinal IgG4-HP. METHODS The authors present a case of spinal IgG4-HP with a systematic review of the literature according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Relevant studies (up to April 2020) that reported patients with spinal IgG4-HP, based on the criteria of Japan College of Rheumatology, were identified from the PubMed and Cochrane Library databases. RESULTS This systematic review identified 33 patients, including the present case, of whom 21 were males and 12 were females. The mean value of age was 51.2 (±12.6) years. Eight patients had systemic involvement. Additionally, among 33 patients, 13 patients had an elevated serum IgG4. Surgery was performed in 31 patients. Steroid therapy alone and steroid therapy with immunosuppressants were effective in 94% and 100% of the cases, respectively. Furthermore, 31 out of 33 patients reported improved outcomes, 1 patient died due to infection and in 2 patients the data were not available. CONCLUSIONS Spinal IgG4-HP is a rare entity. Additionally, it should be considered in the differential diagnosis of space-occupying lesions around the spinal cord. Histopathology with immunohistochemistry results provides the most reliable evidence for diagnosis. Steroid therapy is the first-line of treatment. Surgical decompression may be required in patients presenting with nerve root and/or spinal cord compression. Long term follow-up is necessary for patients with spinal IgG4-HP.