title: [Central nervous system in IgG4-related disease: case report and literature review].
abstract: BACKGROUND
  IgG4-related disease is a recently described multisystemic clinical entity that can occur with different clinical manifestations. The most often affected organs are the pancreas, bile duct and salivary glands, with unusual central nervous system affection.
  
  
  METHODS
  A 33 year old woman who presented with cognitive impairment, hallucinations, headache, convulsive syndrome, maxillary sinus inflammation with bone involvement and evidence of pachymeningitis and panhypopytuirarism with meningeal biopsy that confirmed IgG4-related disease, after ruling out secondary causes. Treatment was started with steroids and azathioprine without relapses after 12 months follow-up.
  
  
  CONCLUSIONS
  IgG4-related disease should be considered in cases of hypertrophic pachymeningitis and hypophysitis especially when no other cause has been found, even if they are not accompanied by other systemic disease manifestations, having ruled out other common causes. The treatment of choice is glucocorticoids and it could be needed to add another immuno-suppressant agent as steroid sparing and to prevent relapses. Prospective studies are needed to evaluate the different clinical and paraclinical manifestations and to establish the results of long-term treatment.
authors.affiliation: Hospital Universitario de San Vicente Fundacion, Medellin, Colombia.|Universidad de Antioquia, Medellin, Colombia.
authors.forename: A L
authors.initials: AL
authors.lastname: Vanegas-Garcia
authors.affiliation: Universidad de Antioquia, Medellin, Colombia.
authors.forename: Y
authors.initials: Y
authors.lastname: Calle-Lopez
authors.affiliation: Universidad de Antioquia, Medellin, Colombia.
authors.forename: C H
authors.initials: CH
authors.lastname: Zapata
authors.affiliation: Universidad de Antioquia, Medellin, Colombia.
authors.forename: D M
authors.initials: DM
authors.lastname: Alvarez-Espinal
authors.affiliation: Universidad de Antioquia, Medellin, Colombia.
authors.forename: Y A
authors.initials: YA
authors.lastname: Saavedra-Gonzalez
authors.affiliation: Universidad de Antioquia, Medellin, Colombia.
authors.forename: J C
authors.initials: JC
authors.lastname: Arango-Viana
mesh_terms: D000328:Adult; D020274:Autoimmune Diseases of the Nervous System / Q000175:diagnosis* / Q000473:pathology; D002490:Central Nervous System / Q000503:physiopathology*; D005260:Female; D006261:Headache / Q000209:etiology; D006801:Humans; D006984:Hypertrophy; D007074:Immunoglobulin G / Q000097:blood*; D007249:Inflammation / Q000209:etiology; D008581:Meningitis / Q000209:etiology; D012640:Seizures / Q000209:etiology
chemical_list: D007074:Immunoglobulin G