Abstract

Introduction

Granulomatosis polyangitis (GPA), formerly known as Wegner granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. GPA is one of the ANCA-associated vasculitides and has a predilection for the upper and lower respiratory tracts and the kidneys. Although most cases of WG occur in adults with a peak age of presentation 64-75 years, it does develop inchildren, with a mean age at diagnosis of 14 yr¹. In children, the estimated incidence is approximately 0.1:100,000². There is a female predominance of 3-4: 1. Its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels.

Case Report

We present a case of a17yrs female adolescent, who presented with fever for 1 month and cough for 15 days. Fever was high grade, max 104.f daily spikes. There was history of productive cough associated with one episode of hemoptysis. On general physical examination, there were some hyper-pigmented lesions over both arms and legs. Rest of the systemic examination was normal. Hearing and eye evaluation was also normal. On investigations blood counts were normal but ESR was highly elevated upto 130mm/Ist hr, RFTs/LFTs/Electrolytes were normal. Urine shows hematuria with 250RBCs per microlitre. Chest xray showed multiple dilated cystic lesions in bilateral lung fields (as shown in figure 1). CT scan showed multiple cavitatory lesions in B/L lung fields with patches of consolidation in B/L upper lobes (figure 2-4).