*Corresponding author: Sodhi Rakhee, Department of Pulmonary Medicine, Himalayan Institute of Medical Sciences, Dehradun. Tel: 07579281136; Email: rakhee.sodhi@gmail.com © 2017 mums.ac.ir All rights reserved. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. An Unusual Presentation of Granulomatosis with Polyangiitis Sodhi Rakhee 1* , Khanduri Sushant 2 , Jethani Varuna 3 , Shirazi Nadia 4 , Kumar Manoj 5 1 Pulmonologist, Department of Pulmonary Medicine, Himalayan Institute of Medical Sciences, Dehradu, Indian 2 Critical Care Medicine, Department of Critical Care Medicine, Himalayan Insitute of Medical Sciences, Dehradu, Indian 3 Senior Resident, Department of Pulmonary Medicine, Himalayan Institute of Medical Sciences, Dehradun, Indian 4 Pathologist, Department of Pathology, Himalayan Institute of Medical Sciences, Dehradun, Indian 5 Resident, Department of Pulmonary Medicine, Himalayan Institute of Medical Sciences, Dehradun, Indian A R T I C L E I N F O A B S T R A C T Article type: Case Report The granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a pauci-immune vasculitis involving small- and medium- sized blood vessels. Classically, the patients inflicted with this disease present with a triad of necrotizing granulomatous inflammation of the respiratory tract, cutaneous necrotizing vasculitis, and glomerulonephritis (GN). The antinuclear (ANA) and anti-neutrophil cytoplasmic antibodies (ANCA) are considered as relatively specific markers of the disease. Herein, we presented the case of a patient who initially presented with sepsis but did not respond to the conventional antibiotics and later diagnosed with GPA. Article history: Received: 14 Jun 2017 Revised: 27 Jul 2017 Accepted: 2 Aug 2017 Keywords: Antinuclear Antibody Anti-Neutrophil Cytoplasmic Antibody Granulomatosis With Polyangiitis Sepsis ►Please cite this paper as: Rakhee S, Sushant Kh, Varuna J, Shirazi N, Manoj K. An Unusual Presentation of Granulomatosis with Polyangiitis. J Cardiothorac Med. 2017; 5(3): 204-207. Introduction The granulomatosis with polyangiitis (GPA) is a systemic vasculitis, which is characterized by destructive granulomatous lesions in the upper and lower respiratory tract and necrotizing crescentic GN. All the small- and medium-sized vessels can be affected; consequently, many potential clinical manifestations may be presented (1). It is believed that the disease begins as a localized respiratory tract granulomatosis, which then generalizes into a vasculitis that affects small and medium-sized vessels (2). Patient presentation varies and depends on the organ system affected. Some patients present with chronic nasal obstruction, which may be misdiagnosed as chronic sinusitis; others may present with overt acute renal or respiratory failure. Patients with pulmonary involvement often complain of cough with or without Hemoptysis, dyspnea, fever, and chest pain (2). We present a case which initially presented as non resolving pneumonia with sepsis and later diagnosed as GPA with preserved renal function. Case presentation A 35-year-old female with no history of chronic diseases was referred to our institute with the complaints of generalized weakness, fever, and dyspnea having been occurred 30, 15, and 5 days before admission, respectively. Additionally, while taking the history, she complained of an ulcer on her right breast emerging from 15 days ago. The vital signs of the patient were reported, rendering a pulse rate (PR) of about 130 bpm, blood pressure (BP) of almost 110 over 80 mmHg, and oxygen saturation level (SpO2) of 94% during the inhalation of room air. A 5×5 cm dry ulcer was