SESSION TITLE: Miscellaneous Student/Resident Case Report Posters I
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Granulomatosis with Polyangitis (GPA) is an autoimmune disease of unknown etiology defined by necrotizing granulomatous inflammation and pauci-immune vasculitis in small and medium sized blood vessels. GPA falls under the category of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides and is associated with anti-proteinase 3 (PR3)-ANCA.
CASE PRESENTATION: A 44 year old Caucasian female with history of asthma, rhinitis and generalized joint pains presented with recurrent dyspnea. She was treated for suspected pneumonia two months prior. CT angiogram (CTA) showed diffuse bilateral pulmonary infiltrates and a large left mainstem endobronchial mass, confirmed as carcinoid tumor on biopsy. Video assisted thoracoscopy revealed an inflamed right lung with diffuse alveolar hemorrhage. She improved with ceftriaxone/levofloxacin and was discharged on high dose prednisone with a prolonged taper. She presented six weeks later with worsening dyspnea and a palpable petechial rash across both lower limbs. Chest x-ray (CXR) and CTA were consistent for bilateral ground glass opacities. Skin biopsy revealed leukocytoclasitc vasculitis. Serology was positive only for c-ANCA. CRP and ESR were mildly elevated. She was treated for suspected post-obstructive pneumonia, but presented two weeks later with worsening dyspnea, fever and a non-productive cough. She was hypoxic with bilateral, diffuse rhonchi on examination. CXR showed stable bilateral pulmonary infiltrates. Vancomycin/Zosyn/Tobramycin were started, along with Octreotide for suspected carcinoid exacerbation, however she continued to deteriorate necessitating intubation. Bronchoscopy revealed diffuse alveolar hemorrhage. Pan-culture was negative. Urinalysis showed 54 red blood cells/HPF, with mild elevation in creatinine. CTA showed worsening pulmonary ground glass opacities. Anti-glomerular basement membrane and myeloperoxidase antibodies were negative, but PR3 antibody was positive. She was started on high dose methylprednisone, induction cyclophosphamide and plasmapheresis with dramatic improvement. She was discharged on prednisone to be followed with oral cyclophosphamide maintenance thereafter.
DISCUSSION: GPA poses a diagnostic challenge as it may mimic many disorders, particularly in early disease. In this case the clinical picture was disguised by a concomitant bronchial carcinoma and post-obstructive pneumonia. However, the combination of ANCA-specific serology and vasculitis on histopathology fulfilled criteria to diagnose GPA. Furthermore, transitioning treatment to focus on immune suppression rather than combating a suspected infection yielded a substantial therapeutic outcome.
CONCLUSIONS: Progression to combine clinical manifestations, imaging, serology and histology has made GPA a less elusive diagnosis
Reference #1: Pamela L.K. Lutalo, David P. D’Cruz. The Journal of Autoimmunity (2014) 1-5. Diagnosis and Classification of Granulomatosis with Polyangiitis (aka Wegener’s Granulomatosis)
DISCLOSURE: The following authors have nothing to disclose: Robert Ali, Candice Baldeo, Jesse Onenykwe, Cristian Landa
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