SADDLE NOSE DEFORMITY FROM EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: THE VERY FIRST CASE

Abstract

SESSION TITLE: Pulmonary Pathology SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/20/2019 01:00 pm - 02:00 pm INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem vasculitic disorder that predominantly affects medium and small sized blood vessels. EGPA belongs to a group of vasculitides known as anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Upper airway involvement is seen in all ANCA associated vasculitides, but destructive upper airway disease has never been reported in patients with EGPA. CASE PRESENTATION: A 50-year-old female with a history of difficult to control asthma for more than 15 years and chronic rhinitis presented to the hospital with severe jaw and ear pain. Vital signs on admission were unremarkable. Physical examination revealed bilateral mandibular tenderness and a normal appearing nasal mucosa. A CT scan of her neck with contrast demonstrated left maxillary sinus thickening and multiple nodules bilaterally at the lung apices. CT scan of the chest revealed bilateral nodules. A clinical diagnosis of EGPA was made and serologic work up was positive for perinuclear ANCA (P-ANCA) and Anti- myeloperoxidase (MPO-ANCA) antibody. Open lung biopsy was performed, and histopathology was consistent with necrotizing granulomatous vasculitis with extravascular eosinophilic infiltrate. She was treated with prednisone and azathioprine. Later on, she developed nonischemic cardiomyopathy. She was started on IV cyclophosphamide with good response. During an office visit, the patient was noted to have depressed nasal bridge with erythematous nasal mucosa. The patient underwent reconstruction of her nasal bridge and currently doing well. DISCUSSION: EGPA most commonly involves upper airway, peripheral nerves, skin and lungs. Destructive upper airway disease, such as saddle nose deformity (SND) is a well-known complication of other AAV, especially granulomatosis with polyangiitis (GPA) but has never been reported in EGPA patients. Other causes of SND include relapsing polychondritis, granulomatous infection, lymphomatoid granulomatosis, lymphoma, carcinoma and trauma. Ear, nose and throat (ENT) involvement is common with EGPA with a reported incidence of 48% on presentation. The most common symptoms include rhinitis, non-erosive sinusitis and nasal polyposis. According to five factors scoring (FFS) system presence of ENT symptoms is considered to convey a better prognosis. It is possible that the SND in our patient was a manifestation of severe systemic inflammation and developed slowly over a 2-year period. The severity of AAV have been proposed to be related to the type of autoantibody rather than the individual vasculitic syndrome. Presence of anti-proteinase 3 antibody (PR3-ANCA) is associated with worse disease severity and more chances of relapse. CONCLUSIONS: Erosive upper airway disease from EGPA has never been reported. We are reporting the first case of SND from EGPA with the hope to enrich the medical literature and encourage further research. Reference #1: Sinico RA, Bottero P. Churg-Strauss angiitis. Best practice & research Clinical rheumatology. 2009;23(3):355-66. DISCLOSURES: No relevant relationships by Scott Beegle, source=Web Response No relevant relationships by Biplab Kumar Saha, source=Web Response No relevant relationships by Aditi Saha, source=Web Response No relevant relationships by Boris Shkolnik, source=Web Response