Sniffles, Hemoptysis, and Headache That Just Would Not Go Away

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 4 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Granulomatosis with polyangiitis (GPA), previously recognized as Wegener’s granulomatosis, is part of group of antineutrophil cytoplasmic autoantibody (ANCA) associated vasculitides (AAV). Some of the clinical criteria for diagnosis include: nasal, or oral inflammation, chest radiographs with the presence of nodules/infiltrates, abnormal urinary sedimentation with microscopic hematuria, and lastly granulomatous inflammation on biopsy of an affected artery. CASE PRESENTATION: A 63 year old hispanic female with a past medical history of hypertension, type II diabetes mellitus who presented for a 3 month history of left sided facial droop, and headache. Patient reported that headache was initially holofrontal, but progressed to include left ear, and subsequent blurry vision. Patient was prescribed cefdinir, and prednisone taper for presumed infection. Patient had associated dysphagia, 20 pound weight loss, low grade fevers, night sweats, intermittent episodes of hemoptysis, and persistent vaginal bleeding for the preceding 2 weeks. On exam, she was afebrile and HEENT exam was significant for left sided facial droop, poor dentition, and cervical lymphadenopathy. Gynecology noted patient to have large cervical mass. Patient started on broad spectrum intravenous antibiotics. CT head/sinus/neck/chest were remarkable for 1 cm intra-axial lesion in right putamen, chronic right maxillary sinusitis, with prominent lymph nodes within anterior cervical, mediastinal regions, left parotid abscess, and multiple spiculated cavitary/noncavitary lung lesions. 2D echocardiogram had no vegetations. Erythrocyte sedimentation rate was 94 and C-reactive protein was elevated 26.87. Patient was started on oral prednisone taper, and antibiotics for Bell’s palsy, and parotiditis. Patient received lumbar puncture with cerebral spinal fluid pleocytosis. Bronchoscopy noted fungating mass with necrotic base, and negative cultures. Cervical mass biopsy noted necrotic cells with no mention of malignancy. Autoantibody to proteinase-3 (PR-3 (c-ANCA)) level was 17.4 (reference range <0.1). Urinalysis was significant for proteinuria, hematuria, and glycosuria. Patient was started on high dose methylprednisone, and plasmapheresis with slight improvement in Bell’s palsy, and systemic symptoms. DISCUSSION: Positive C-ANCA is detected in > 85% of patients with active disease. GPA generally presents in older Caucasian adults (40-65 years old) with no gender predominance. If left untreated GPA has a poor prognosis with up to 90% mortality rate. CONCLUSIONS: GPA has been seldom described in literature with symptom presentation including: progressive ipsilateral peripheral facial nerve paralysis, parotiditis, mononeuritis multiplex, cervical granulomas, hemoptysis, along with progressive kidney injury. Reference #1: Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Ann Rheum Dis. 2011;70(4):704. DISCLOSURE: The following authors have nothing to disclose: Emmanuel Bassily, Daniel Mathew, Vindya Gunawardena, Jibran Khan, Sean Verma, Chak Sriaroon No Product/Research Disclosure Information