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Neurol Neuroimmunol Neuroinflamm. 2019 May 7;6(4):e568. doi: 10.1212/NXI.0000000000000568. eCollection 2019 Jul.

Immunoglobulin G4-related hypertrophic pachymeningitis: A case-oriented review.

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Service de Médecine Interne (M.L., N.M.), Hôpital l'Archet 1, Centre Hospitalier Universitaire de Nice, Université Côte d'Azur; Service de Cytologie Pathologique (F.B.-V.), Hôpital l'Archet 1, Centre Hospitalier Universitaire de Nice, Université Côte d'Azur; and Service de Neurologie (M.C., S.B., C.G., V.B., C.L.-F.), Service de Radiologie (L.M.), Service de Radiologie Interventionnelle (J.S.), and Service de Neurochirurgie (D.F.), Hôpital Pasteur 2, Centre Hospitalier Universitaire de Nice, Université Côte d'Azur, France.



Meningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP).


Two IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and case-series of histologically proven IgG4-HP that were published in the PubMed-NCBI database.


Forty-two case reports and 5 case-series were studied (60 patients, 20 women). The median age was 53. Eighteen patients had systemic involvement and 24 had single-organ IgG4-HP. Fifty-five percent of patients had an elevated serum IgG4. Treatment was surgical in 20/53 cases. Steroid therapy and immunosuppressors were effective in 85% and more than 90% of the cases, respectively. The rate of disease relapse was 42.1% after steroid therapy was discontinued.


IgG4-HP is characterized by the lack of extra-neurologic organ-involvement and systemic signs. Histopathologic studies should be performed as it is crucial for diagnosis because serum markers are rarely informative. 18F-FDG positon tomography can be useful to characterize systemic forms. There is no specific CSF marker for IgG4-HP and the diagnostic value of CSF IgG4 levels needs to be studied with larger samples. We provide a treatment algorithm for IgG4-HP. Such treatment strategies rely on early surgery, steroids, and early immunosuppressive therapy to prevent neurologic complications.

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