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

OBJECTIVEMeningeal 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). METHODSTwo IgG4-H...

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Published in:Neurology : neuroimmunology & neuroinflammation 2019-07, Vol.6 (4), p.e568-e568
Main Authors: Levraut, Michaël, Cohen, Mikaël, Bresch, Saskia, Giordana, Caroline, Burel-Vandenbos, Fanny, Mondot, Lydiane, Sedat, Jacques, Fontaine, Denys, Bourg, Véronique, Martis, Nihal, Lebrun-Frenay, Christine
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Language:English
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Summary:OBJECTIVEMeningeal 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). METHODSTwo 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. RESULTSForty-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. DISCUSSION/CONCLUSIONIgG4-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.
ISSN:2332-7812
2332-7812
DOI:10.1212/NXI.0000000000000568