ANCA-Associated Vasculitis - Revisited, and its Association with Autoimmune Thyroid Disease

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of uncommon diseases. Given its rarity and multisystem clinical presentation which are usually serious and potentially fatal, prompt recognition and early treatment are of utmost importance. We report a case of AAV that presented with digital infarcts, cutaneous vasculitis, rapidly progressive glomerulonephritis, mononeuritis multiplex, eosinophilia and positive myeloperoxidase (MPO)-ANCA antibody. Apart from renal damage, there was complete recovery in other systems following intravenous cyclophosphamide and high-dose glucocorticoids albeit the response was delayed. This response obviates the need for second-line therapy with newer agent such as rituximab (anti-CD20 monoclonal antibody). We would also like to highlight that this is the first case of AAV that is associated with autoimmune thyroid disease to be reported from Malaysia.