IgG4-related disease (IgG4-RD) is a systemic inflammatory condition marked by tissue infiltration of IgG4-positive plasma cells, often resulting in fibrosis. While IgG4-RD commonly affects organs such as the pancreas and salivary glands, central nervous system involvement, particularly hypertrophic pachymeningitis (HP), is rare and can present unique clinical challenges. This case report describes a 67-year-old male with IgG4-related hypertrophic pachymeningitis complicated by recurrent cerebral venous thrombosis (CVT), a rarely reported association. The patient initially responded to high-dose corticosteroids, but experienced symptom relapse upon tapering, necessitating treatment with rituximab for sustained disease control.In addition to presenting this case, we provide a comprehensive review of the literature on IgG4-RHP with CVT, analyzing clinical presentation, anatomical involvement, IgG4 serum levels, histopathological findings, treatment strategies, and outcomes across reported cases. Our analysis highlights the challenges of managing recurrent CVT in IgG4-RHP and underscores the importance of immunosuppressive therapy, particularly B-cell depletion, in achieving long-term disease stability. This case and literature review offer insights into the pathophysiology, diagnostic approach, and therapeutic considerations for this complex and rare manifestation of IgG4-RD.
Wang et al. (Wed,) studied this question.