MPO and connective tissue disorder: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), particularly myeloperoxidase (MPO)-ANCA-positive granulomatosis with polyangiitis (GPA), rarely present with acute arterial digital ischemia or necrosis.<i> </i> We report a 68-year-old woman with a history of seropositive rheumatoid arthritis, suspected connective tissue disease (CTD)-associated interstitial lung disease, scleromalacia perforans, and osteoporosis who presented with acute ischemia of the left hallux that progressed to involve multiple digits despite therapeutic anticoagulation.