Granulomatosis with polyangiitis: recurrence presenting as ependimoplexitis

Clin Exp Rheumatol. 2014 May-Jun;32(3 Suppl 82):S70-2. Epub 2014 Mar 17.

Abstract

A 55-year-old man with granulomatosis with polyangiitis (GPA) developed continuous parietal headache, malaise, nasal crusting and dry cough. Neurological exam revealed only left hand hypoesthesia in 4th and 5th finger. Brain MRI showed enlarged right choroid plexus, hyperintense periventricular white matter, thalami and right side of corpus callosum. The suspected diagnosis was ependimoplexitis due to GPA, the patient received three 500 mg methylprednisolone pulses followed by 1 mg/kg of meprednisone with gradual tapering and was switched to oral cyclophosphamide. He had complete resolution of headache. An MRI following this treatment for relapse revealed only minimal ependimal changes.

Publication types

  • Case Reports

MeSH terms

  • Choroid Plexus / pathology*
  • Cyclophosphamide / administration & dosage*
  • Diagnosis, Differential
  • Dose-Response Relationship, Drug
  • Drug Administration Schedule
  • Granulomatosis with Polyangiitis* / complications
  • Granulomatosis with Polyangiitis* / diagnosis
  • Granulomatosis with Polyangiitis* / drug therapy
  • Granulomatosis with Polyangiitis* / immunology
  • Granulomatosis with Polyangiitis* / physiopathology
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Magnetic Resonance Imaging / methods
  • Male
  • Methylprednisolone / administration & dosage*
  • Middle Aged
  • Neurologic Examination / methods
  • Tomography, X-Ray Computed / methods
  • Treatment Outcome
  • Vasculitis, Central Nervous System* / diagnosis
  • Vasculitis, Central Nervous System* / etiology

Substances

  • Immunosuppressive Agents
  • Cyclophosphamide
  • Methylprednisolone