title: A multi-centre case series investigating the aetiology of hypertrophic pachymeningitis with orbital inflammation.
abstract: BACKGROUND
  To describe our attempt in establishing a definitive diagnosis in patients with hypertrophic pachymeningitis in combination with orbital inflammatory disease and report on the outcome.
  
  
  METHODS
  This was a retrospective case series of all patients presenting with hypertrophic pachymeningitis in association with orbital inflammation in 4 centres. Ophthalmic and neurological examination data, laboratory data, histology data, treatment plans and clinical outcome data were recorded. Patients underwent orbital/brain computed tomography and magnetic resonance imaging.
  
  
  RESULTS
  Six patients were identified; the median age was 46.5 years. Headache was the commonest presenting symptom, followed by diplopia and reduced visual acuity. Three patients underwent orbital biopsy, 1 patient underwent dura mater biopsy, 1 patient underwent both and 1 patient underwent nasal biopsy. Four patients were diagnosed with Wegener granulomatosis and 2 patients with tuberculosis. Corticosteroid therapy was initiated in 4 patients, with steroid-sparing drugs added later. Two patients received anti-tuberculosis treatment and 1 patient was commenced on pulsed cyclophosphamide. On follow-up, 1 patient required an exenteration for a painful blind eye and 1 patient's visual acuity remained at no perception to light. One patient had complete resolution of symptoms on treatment, 1 patient had persistent reduced visual acuity and 1 patient was lost to follow-up.
  
  
  CONCLUSIONS
  We postulate that the combination of orbital inflammation and pachymeningitis is strongly suggestive of Wegener granulomatosis, although it may take a number of years to confirm. Tuberculosis should also be considered.
authors.affiliation: Discipline of Ophthalmology & Visual Sciences, University of Adelaide, Adelaide, SA 5000, Australia. pscan05@yahoo.co.uk
authors.forename: Paul S
authors.initials: PS
authors.lastname: Cannon
authors.forename: Antonio A V
authors.initials: AA
authors.lastname: Cruz
authors.forename: Carolina T
authors.initials: CT
authors.lastname: Pinto
authors.forename: Dante A
authors.initials: DA
authors.lastname: Mastropietro
authors.forename: Fernando
authors.initials: F
authors.lastname: Chahud
authors.forename: Jurij R
authors.initials: JR
authors.lastname: Bilyk
authors.forename: Dinesh
authors.initials: D
authors.lastname: Selva
authors.forename: Venkatesh C
authors.initials: VC
authors.lastname: Prabhakaran
mesh_terms: D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D000995:Antitubercular Agents / Q000627:therapeutic use; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology*; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D006984:Hypertrophy; D008279:Magnetic Resonance Imaging; D008297:Male; D008581:Meningitis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology*; D008875:Middle Aged; D054517:Orbital Cellulitis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014374:Tuberculin Test; D014392:Tuberculosis, Ocular / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D000995:Antitubercular Agents; D005938:Glucocorticoids; D003520:Cyclophosphamide