Proteinase 3 (PR3)-Positive Perinuclear Anti-neutrophil Cytoplasmic Antibodies (pANCA) Vasculitis With Concurrent...

Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides are a group of rare diseases that cause inflammation of small to medium vessels. They comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). As the signs and symptoms are varied, the diagnosis of vasculitis is complex and challenging; however, there are type-specific clinical presentations that can be factored into the existing classification criteria. The difficulty faced in diagnosis is compounded due to ANCA being present in other chronic illnesses. While ANCA vasculitis may be triggered by infection, it is rarely associated with invasive aspergillosis. We present a case of proteinase 3 (PR3)-positive vasculitis with concurrent invasive aspergillosis in a 68-year-old male in whom both the clinical presentation and immunochemical picture were atypical. However, he responded well to treatment with immunosuppression. This report highlights the complexities in the diagnosis of vasculitis and the limitations of existing diagnosis and inclusion criteria.