028. PACHYMENINGITIS AS THE INITIAL PRESENTATION OF GRANULOMATOSIS WITH POLYANGIITIS: A CASE SERIES OF FOUR PATIENTS

Abstract

Background: Granulomatosis with polyangiitis (GPA) is the most common of the ANCA-associated vasculitides, with an incidence of 11/million. 20% of patients manifest with neurological symptoms, typically as mononeuritis multiplex, with pachymeningitis as one of its rarer forms of presentation. Method(s): The departmental database was searched to identify patients with diagnosis of GPA. Electronic patient records were reviewed to select patients presenting with pachymeningitis. Result(s): Case 1: 48-year-old male with 2-year history of conjunctivitis, hearing loss, diplopia and headaches. MRI/CT of the head and sinuses and biopsy consistent with pachymeningitis. pANCA MPO 17U/mL. The patient failed 2 courses of iv methylprednisolone and iv cyclophosphamide with worsening symptoms and extension of disease on MRI. Responded to Rituximab followed by Azathioprine, remaining asymptomatic at 3-year follow-up with minimal enhancement on MRI. Case 2: 19-year-old female with one-year history of parotiditis, Bell's phenomenon, nasal bridge collapse, epistaxis and hearing loss. CT of the sinuses and a nasal biopsy consistent with GPA. cANCA PR3 9U/mL, MPO 7U/mL. Responded to cyclophosphamide iv and Prednisolone followed by Azathioprine. Flared after 28 months with blood-stained tears, rise in PR3 titres and radiological progression. Responded to a second course of cyclophosphamide iv followed by mycophenolate mofetil (MMF) and prednisolone. At 6-years of follow-up she remains well with residual changes on MRI, and sequelae of facial palsy and hearing loss. Case 3: 23 year-old male with several months of multiple cranial nerve palsies (II, III, IV, VI and VII) and headaches. cANCA pattern unspecific. MRI showed pachymeningitis. Meningeal biopsy confirmed GPA. Failed 2 courses of high dose steroids, iv cyclophosphamide but achieved remission on oral cyclophosphamide for 3 months (lymphopaenia). Second remission with Methotrexate (MTX) but flared after 20 months (sinusitis and headaches). Failed combination with Leflunomide (weight loss), Azathioprine (lymphopaenia, anaemia) and responded to combination with MMF for 15 months (pancreatitis). Refused Rituximab and continues on MTX and dependent on high dose steroids, with sequelae partial blindness, chronic sinusitis and Horner syndrome. Case 4: 77 year-old female with 1 month history of headaches and 2 TIAs. MRI showed pachymeningitis, cANCA PR3 532U/mL. Incidental pulmonary nodules on PET. Responded to Cyclophosphamide IV and Prednisolone, followed by Azathioprine. After 3 months worsening headache, rise in cANCA titres and progression of meningeal enhancement on MRI. Responded to Rituximab followed by MMF which was switched to Azathioprine due to recurrent UTIs. At 24 months of follow up is well only on Azathioprine. Conclusion(s): Despite localized disease all patients needed aggressive immunosuppression with Cyclophosphamide or Rituximab and high doses of steroids. 3 patients flared after induction of remission, requiring re-treatment with Cyclophosphamide, Rituximab or DMARD combination. There was no progression to other organ involvement.