Abstract

Granulomatous with Polyangiitis (GPA) rarely presents as the peripheral vascular disease is an early manifestation. Most of the cases of GPA have ANCA positive (90%), and 10% are ANCA negative. A case of 32 yr old female who presented with painful gangrene of digits of both upper limb and lower limb and purpura on the lower leg and nasal crusting. CT angiogram of upper limbs shown radial and ulnar cut off and lower limbs narrowed lumen and non-visualisation of the posterior tibial artery. HRCT chest shows focal consolidation and cavitary lesions and soft tissue nodules in the right middle and lower lobes suggestive of granulomatous etiology. biopsy of skin shown vessels occluded with thrombi and transmural inflammation of vessel wall and renal involvement in the form of albuminuria, ANCA were negative. In view of clinical features and HRCT findings and biopsy reports diagnosed as ANCA negative GPA. According to ACR criteria, nasal involvement, lower respiratory tract involvement, renal involvement, and ANCA positivity, 2/4 is enough to diagnose GPA. In our case, three of them are seen. Patient was started on steroids and cyclophosphamide therapy; there was no progression of gangrene, and patient shown symptomatic improvement after treatment. There were less than 20 reported cases of GPA presenting as peripheral vascular disease. Hence GPA should be considered as a possibility in a patient with peripheral vascular disease.