P-ANCA POSITIVE GRANULOMATOSIS WITH POLYANGIITIS

Abstract

SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: It is challenging to diagnose ANCA associated vasculitis (AAV) and specify the subtype of AAV due to the overlap in symptoms and lab findings. Diagnosis can be supported by positive ANCA titer or a biopsy that confirms vasculitis present [2]. The typical ANCA pattern associated with GPA is c-ANCA. GPA is associated with P-ANCA (MPO positive) in less than 20% of the cases and approximately 10 % are ANCA negative [1] . Otorhinolaryngologic symptoms may be the initial clinical manifestation of GPA because the upper respiratory tract is involved in 70–100% of cases. Both patients of GPA and MPA can present with tracheal complications such as tracheal stenosis although it is more common in GPA. Glottic or supraglottic stenosis is a rare manifestation of AAV [1]. We present this rare case of p ANCA associated GPA presenting as stridor. CASE PRESENTATION: A 62-year-old female presented with cough, dyspnea, weight loss and tongue sores. She had significant stridor on physical exam. CT of the neck and soft tissues showed aryepiglottic edema, subglottic stenosis and pan sinus disease. Laryngoscopy in the ED and showed poor true vocal cord mobility. She then underwent tracheostomy, in addition to tongue biopsy. Direct laryngoscopy confirmed tight vocal cords, with no evidence of subglottic stenosis and normal arytenoids. Despite that the patient had increasing oxygen requirements. Her chest CT showed increased bilateral lung opacities. Bronchoscopy showed evidence of diffuse alveolar hemorrhage and granulomatous appearance of the trachea and the main stem bronchi. Work up for vasculitis was in process, while the tongue biopsy confirmed vascular inflammation and fibrinoid necrosis. later we had positive P-ANCA titer of >1:10240, MPO 25 AU/ML, negative PR3 and Anti GBM. she was started on pulse methylprednisolone and rituximab in addition to five sessions of plasmapheresis. DISCUSSION: GPA is distinguished from MPA by the absence of granulomas in the latter. Most involved organs are lungs, kidneys and ear, nose and throat. ENT involvement occurs in more than 50% of patients with AAV and can manifest as simple crusting, chronic sinusitis and rhinorrhea, to as severe as hearing loss or oral or nasal ulcers. Early diagnosis is crucial to prevent long term complications.[1] Tracheobronchial involvement is less common in GPA and would usually present as subglottic stenosis. [1], even rare is vocal cord paralysis. Our patient was diagnosed with AAV related vocal cord paralysis, which manifested as stridor. Severe active GPA can be treated with Glucocorticoids along with chemotherapeutic agents i.e cyclophosphamide or Rituximab infusion. Plasmapheresis is also helpful as it helps remove ANCA from the blood in addition to other inflammatory mediators [3]. CONCLUSIONS: Vocal cord paralysis is a rare presenting symptom for GPA, it is important to recognize it as early treatment affects the prognosis of the patient. Reference #1: 1.Greco A, Marinelli C, Fusconi M, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2016;29(2):151–159. doi:10.1177/0394632015617063 Reference #2: 2.Wojciechowska J, Krajewski W, Krajewski P, Kręcicki T. Granulomatosis With Polyangiitis in Otolaryngologist Practice: A Review of Current Knowledge. Clin Exp Otorhinolaryngol. 2016;9(1):8–13. doi:10.21053/ceo.2016.9.1.8 Reference #3: 3.Walsh M, Merkel PA, Peh CA, et al. Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial. Trials. 2013;14:73. Published 2013 Mar 14. doi:10.1186/1745-6215-14-73 DISCLOSURES: No relevant relationships by Reena Bansal, source=Web Response No relevant relationships by Lana Hasan, source=Web Response No relevant relationships by Rajani Jagana, source=Web Response No relevant relationships by Natalya Shlyakhova, source=Web Response