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

Published on Jul 1, 2019in Neuroimmunology and Neuroinflammation
· DOI :10.1212/nxi.0000000000000568
M. Levraut , Mikael Cohen9
Estimated H-index: 9
+ 8 AuthorsChristine Lebrun-Frenay16
Estimated H-index: 16
Objective 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). Methods 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. Results 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. Discussion/conclusion 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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#1Mahmoud A. AbdelRazek (Harvard University)H-Index: 1
#2Nagagopal Venna (Harvard University)H-Index: 16
Last.John H. Stone (Harvard University)H-Index: 67
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#1Zachary S. Wallace (Harvard University)H-Index: 17
#2Hamid Mattoo (MIT: Massachusetts Institute of Technology)H-Index: 16
Last.John H. Stone (Harvard University)H-Index: 67
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#1Yu Chen (Peking Union Medical College Hospital)H-Index: 3
#2J.Z. Zhao (Peking Union Medical College Hospital)H-Index: 2
Last.Fengchun Zhang (Peking Union Medical College Hospital)H-Index: 28
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