An Unusual Presentation of Granulomatosis with Polyangiitis (GPA)

BACKGROUND This is a very interesting case of Granulomatosis with Polyangiitis (GPA) presenting with hand ischemia which rapidly evolved into dry gangrene from the involvement of digital arteries. GPA usually affects the small and medium sized blood vessels. GPA affecting muscular artery causing limb ischemia is a rare manifestation. CASE REPORT A 72 years old Caucasian female was sent to our hospital for the evaluation of left hand pain and bluish discoloration, which she noticed for few days. Physical examination was notable for bluish discoloration of left 2nd to 5th fingers, which later evolved, into dry gangrene at the tips. Angiogram revealed ischemia with no evidence of thrombosis. Administration of intra-arterial nitroglycerin resulted in improved blood flow through the radial artery. Labs revealed ESR of 142 mm/hr. C-ANCA titer was 5120, (normal <20) and antiproteinase 3 was 1117 (normal <20) consistent with GPA. CONCLUSIONS GPA usually affects the small and medium sized blood vessels. This case highlights an unusual presentation of GPA manifesting as critical limb ischemia. Given the rarity of this circumstance, a high index of suspicion is necessary in order to initiate proper treatment and limb salvaging intervention.