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A2994 - ""I'm Bleeding from My Eyes"" - Acute Onset Dacryoadenitis, Later Diagnosed with Granulomatosis with Polyangiitis
Author Block: K. Kongpakpaisarn1, K. N. Cao2; 1Internal Medicine, University of South Florida, Tampa, FL, United States, 2University of South Florida - UMSA, Tampa, FL, United States.
Granulomatosis with polyangiitis (GPA) is a rare complex, immune mediated multisystem disease. The most common organs involved are the genitourinary and respiratory systems. Ocular involvement occurs in 50 - 60% of patients. The diagnosis can be challenging and delay of treatment can lead to significant disability or death. We describe a case of acute progressive dacryoadenitis followed by hypercapneic respiratory failure despite high dose steroids that was transferred to our facility for a second opinion.
55 year-old African-American female presented with acute onset dacryoadenitis and hypercapneic respiratory failure requiring BiPAP. Blood cultures, bronchoalveolar fluid cultures and transbronchial biopsies showed nonspecific inflammation without evidence of granulomas. 60 mg of prednisone resulted in slight improvement in periorbital edema. Chest imaging showed perihilar airway thickening with perilymphatic nodularity and narrowing of the distal trachea and main bronchi. MRI of the orbits showed diffuse bilateral enhancement of the lacrimal glands. The patient’s sedimentation rate, high-sensitivity C-reactive protein and eventually, the antineutrophil cytoplasmic autoantibody (ANCA) and proteinase-3 returned positive. She developed progressive respiratory failure that improved significantly with increase in steroids to 1 gram daily. Repeat bronchoscopy with cryobiopsies were performed and revealed right bronchial edema, diffuse alveolar hemorrhage and pathology revealed necrotizing mixed inflammation with associated histiocytic aggregates and leukocytic angiitis. She was able to be discharged on Rituximab and prednisone. However, she returned two months later with progressive weakness and recurrent respiratory failure requiring mechanical support. Repeat bronchoscopy revealed diffuse severe webbing and we utilized argon plasma coagulation and cryosurgical techniques to open the airways.
Granulomatosis with polyangiitis patients usually present with nonspecific symptoms such as fever, cough, dyspnea, malaise, and weight loss. Around 90% of the patients are white and multiple organ involvement is usually seen. Respiratory involvement of GPA includes both upper and lower airways. Clinical manifestations are wide and can include acute rhinosinusitis, tracheal stenosis, interstitial lung disease, granulomatous inflammation and alveolar hemorrhage. As other inflammatory diseases can present with similar symptoms and imaging, it is important to consider and evaluate for possible sarcoidosis, IgG4-related disease, collagen vascular diseases, and other types of vasculitis. If there is a high suspicion of GPA based on clinical findings and ANCA, biopsy is not always needed and treatment should not be delayed. However, in atypical presentations, such as in our patient, biopsies are warranted to rule out alternative causes.