Granulomatous polyangiitis with pachymeningitis: a case report.

A 33-year-old man had 1 month of right-sided headache with multiple right-sided cranial neuropathies. He had a history of recurrent sinusitis, elevated inflammatory markers and positive cytoplasmic anti-neutrophilic cytoplasmic antigens (c-ANCA) anti-proteinase 3 (anti-PR3). MR scans of the brain and cervical spine identified an inflammatory mass at the skull base with hypertrophic pachymeningitis and a sigmoid sinus thrombosis. Cerebrospinal fluid examination excluded infection and a meningeal biopsy confirmed vasculitis. CT scan of the chest showed multiple lung nodules with cavitation. We gave intravenous cyclophosphamide and corticosteroids as induction therapy for granulomatosis with polyangiitis. Unfortunately, during the induction phase, he developed diffuse alveolar haemorrhage and progressive mononeuritis multiplex. We subsequently gave intravenous rituximab and C5a receptor antagonist, avacopan. He recovered well and remains in remission.