Upper-extremity digital ischemia in granulomatosis with polyangiitis

We report a case of granulomatosis with polyangiitis (GPA) in a 48-year-old man that was complicated by upper-extremity digital ischemia. Our patient initially presented with acute kidney injury, nasal and sinus symptoms, and bilateral lower-extremity swelling. He was diagnosed with GPA based on clinical features, findings on renal biopsy, and positive antineutrophilic cytoplasmic antibodies. He was treated with immunosuppression, but during the course of the disease, he developed cyanosis in his bilateral upper extremities. In our case, middle-/large-size arteries (Chapel Hill Consensus Conference nomenclature) in the upper extremities were occluded, and this caused the digital ischemia. Despite treatment with immunosuppression, plasmapheresis, and antithrombotic therapy, our patient's digital ischemia progressed leading to autoamputation of his fingers. Because of the rarity of digital ischemia in GPA, this case presented with many diagnostic and therapeutic challenges.