IgG4-related spinal hypertrophic pachymeningitis: a systematic review.

Abstract

IgG4-related hypertrophic pachymeningitis (IgG4-RHP) is a rare disorder characterized by fibroinflammatory thickening of the cranial or spinal dura mater. IgG4-related spinal hypertrophic pachymeningitis (IgG4-SHP) often mimics spinal space-occupying lesions, complicating diagnosis. Prompt and accurate diagnostic strategies are essential. Our review summarized the clinical features and treatment outcomes of IgG4-SHP to guide clinical diagnosis and management. A systematic review was conducted in accordance with PRISMA recommendations for systematic reviews of case series. PubMed, Scopus, Web of Science, and DOAJ were searched up to October 28, 2025, using the terms "IgG4," "hypertrophic pachymeningitis," and "spine." Inclusion and exclusion criteria were predefined, and reference lists were screened manually for additional eligible studies. A total of 62 patients (36 males, 26 females; mean age 49.2 ± 14.7 years) were reviewed. IgG4-SHP often affects the cervical and thoracic spine and is associated with progressive neurological deficits. More than half of patients with available data had elevated serum IgG4 levels and abnormal cerebrospinal fluid findings, but these laboratory findings were considered adjunctive rather than diagnostic. Glucocorticoid-based therapy and surgical decompression were frequently reported, particularly in cases with marked neural compression; however, comparative treatment efficacy could not be assessed. This review highlights the diagnostic challenges of IgG4-SHP and emphasizes the importance of histopathological confirmation. This systematic review provides a comprehensive summary to date of clinical features, treatment approaches, and outcomes. Our findings emphasize the need for greater awareness and early recognition of this rare but treatable condition.