A Case of ANCA-Associated Vasculitis Presenting with Spontaneous Intracerebral Haemorrhage and Hypertrophic Pachymeningitis

Background: Anti-neutrophil cytoplasmic antibody associated vasculitis is a paucity immune systemic small-vessel vasculitis. Objective: If left untreated it carries substantial burden of morbidity and mortality. We report here a case of ANCA-Associated Vasculitis with spontaneous intra cerebral haemorrhage and hypertrophic pachymeningitis. Materials and Methods: CT scan revealed intra cerebral haemorrhages and a dura based intracranial granuloma. Rheumatoid factor and anti-nuclear antibody were negative. Serum PR3 ANCA tested positive with high titer 77.8 IU/ml (negative < 5) and serum MPO ANCA was 1.5 (negative < 5). There was no renal and pulmonary involvement. Result: Keeping high index of suspicion and approach through clinical, radiological and serological parameters, we could diagnose the patient in a nine-day stay at the hospital and initiate specific therapy. Discussion and Conclusion: Anti-neutrophil cytoplasmic antibody associated vasculitis may present with uncommon manifestations like intracerebral haemorrhage and/or hypertrophic pachymeningitis and a high index of suspicion is required for early diagnosis and prompt treatment to prevent adverse outcomes.