424. Endobronchial Disease in a Patient with ANCA-Associated Vasculitis
Creators: name: Caroline Vo1, 2
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Presentation of Case</strong>: A 60-year-old male lifelong non-smoker presented to the emergency room with frontal headaches and diplopia. He also reported progressive fatigue, cough, and hemoptysis. His medical history included asthma and seropositive rheumatoid arthritis treated with golimumab. He also had chronic rhinosinusitis and had undergone three polypectomies. <strong>Diagnostic Testing:</strong> A chest computed tomography (CT) demonstrated patchy infiltrates, bilateral upper lobe cavitating lesions and peribronchial nodules. Initial bronchoscopy showed diffuse, severe ulceration of the proximal airways with mucosal bleeding involving the upper lobes, lingula and right middle lobe. The bronchoalveolar lavage (BAL) was bloody, but not sequentially hemorrhagic to indicate diffuse alveolar hemorrhage. Laboratory examination revealed an elevated proteinase 3 anti-neutrophil cytoplasmic antibodies (&gt; 8 AI) and rheumatoid factor (116 IU/mL). Creatinine peaked during his admission. Urinalysis showed proteinuria and hematuria. His CBC was unremarkable with a normal eosinophil count. <strong>Differential &amp; Final Diagnosis</strong><strong>:</strong> The differential included infectious trachea-bronchitis and malignancy. Two separate BAL and biopsy samples did not demonstrate infectious etiologies. There was a positive BAL qualitative CMV and HSV polymerase chain reaction, but this was not considered relevant as the patient was not immunocompromised and his clinical presentation was not consistent with a viral pneumonitis. Malignancy was not found on lavage or pathology. Confirmatory testing included a repeat bronchoscopy with examination of nares that showed persistent ulceration, mucosal edema and erythema (Figure 1). Biopsy of the right upper lobe bronchus was revealing for necrotizing granulation inflammation. A subsequent kidney biopsy demonstrated focal segmental proliferative and necrotizing glomerulonephritis, which is consistent with ANCA associated glomerulonephritis. Overall, the patient had a constellation of symptoms consistent with granulomatosis with polyangiitis (GPA). This includes serology (PR3-ANCA positive), pulmonary involvement confirmed on bronchoscopy and CT, nasal polypoid disease, bronchial mucosal inflammation and glomerulonephritis. <strong>Discussion of Management</strong><strong>:</strong> GPA is a small vessel vasculitis characterized by necrotizing, granulomatous inflammation. A systemic auto-immune disease, GPA predominantly affects the upper respiratory tract, pulmonary parenchyma and kidneys. Endobronchial disease in comparison is relatively rare with mostly case reports or series descriptions, but it carries significant morbidity and mortality. While subglottic stenosis has an incidence of 10%, distal endobronchial disease is reported in only 6% of GPA patients. The presence of endobronchial disease should prompt consideration for both immediate (ex. infectious trachea-bronchitis) and long-term complications (ex. tracheal or bronchial stenosis). Our patient was treated with three doses of IV methyl-prednisolone, followed by high-dose oral prednisone. His high risk of relapse with positive PR3-ANCA was in favor of utilizing rituximab as induction therapy. <strong>Disclosures</strong><strong>:</strong> None. *These authors contributed equally to this abstract. <strong>Figure 1</strong><strong>.</strong> A) Anterior nares with ulceration and clots, and B) the right upper lobe bronchus demonstrating erythema, ulceration, and edema with adherent mucous.