title: Steroid-resistant relapsing IgG4-related pachymeningitis treated with methotrexate.
abstract: OBJECTIVE
  IgG4-related disease, which is newly recognized, is characterized by lymphoplasmacytic infiltration with increased IgG4-secreting plasma cells. Although a favorable response to steroids has previously been reported, the durations of follow-up to confirm the long-term benefits and clinical courses were limited. We describe long-term favorable response of oral methotrexate in a patient with IgG4-related pachymeningitis who was resistant to steroid therapy.
  
  
  METHODS
  A patient in his mid-60s with pathologically proven IgG4-related pachymeningitis who was resistant to steroid therapy and experienced an exacerbation of symptoms 4 times is described. Low-dose oral methotrexate induced significant clinical and radiological improvement, with sustained remission of the disease over 2 years without complications.
  
  
  CONCLUSIONS
  The long-term favorable response to oral methotrexate in the current patient suggests that methotrexate is a useful alternative treatment option in patients with IgG4-related pachymeningitis who are resistant to steroid therapy or who experience adverse effects from steroids.
authors: Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors: Jae-Won
authors: JW
authors: Hyun
authors: Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors: Su-Hyun
authors: SH
authors: Kim
authors: Department of Neurosurgery, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors: Heon
authors: H
authors: Yoo
authors: Department of Pathology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors: Eun-Kyung
authors: EK
authors: Hong
authors: Department of Neurology, Kosin University School of Medicine, Busan, Korea.
authors: So-Young
authors: SY
authors: Huh
authors: Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors: Ho Jin
authors: HJ
authors: Kim
mesh_terms: D000284:Administration, Oral; D000368:Aged; D004351:Drug Resistance / Q000187:drug effects* / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008297:Male; D008581:Meningitis / Q000175:diagnosis* / Q000188:drug therapy* / Q000276:immunology; D008727:Methotrexate / Q000008:administration & dosage*; D012008:Recurrence; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D007074:Immunoglobulin G; D013256:Steroids; D008727:Methotrexate