SESSION TITLE: Airway Cases I
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 27, 2013 at 07:30 AM - 08:30 AM
INTRODUCTION: Amyloidosis is a rare disease caused by extracellular deposition of amyloid. Isolated pulmonary amyloidosis requires the absence of systemic amyloidosis and can appear as parenchymal nodules, diffuse parenchymal infiltrates or submucosal deposits in the airways1.
CASE PRESENTATION: 58-year-old healthy male from Peru, lifetime non-smoker, dental laboratory technician developed productive cough, chest pressure and dyspnea. He was hospitalized with left lower lobe pneumonia and treated with broad-spectrum antibiotics. He was readmitted two weeks later for ongoing dyspnea, cough, weight loss and acute renal failure. Chest radiograph showed a persistent left basilar opacity. Computed tomography of the chest revealed endobronchial polypoid lesions and enlarged mediastinal and hilar lymph nodes. PET showed hypermetabolic right paratracheal, subcarinal and bilateral hilar lymphadenopathy. Bronchoscopy demonstrated numerous polyps along the main carina, bronchus intermedius and distal left mainstem bronchus with partial left lower lobe bronchial obstruction. Bronchial brushings and bronchoalveolar lavage were negative for malignant cells; endobronchial biopsy inconclusive. Patient was evaluated at our institution for a second opinion. His vital signs were stable and physical exam was unrevealing. Bronchoscopy confirmed prior findings. EBUS-TBNA of level 4R and 7 lymph nodes, endobronchial debulking of left lower lobe nodules and Nd:YAG laser treatment were performed. Pathology showed AL-type amyloidosis, associated with monotypic plasmacytosis. TBNA of level 7 lymph node was positive for abundant amyloid and negative for malignant cells. Blood work revealed BUN 48, creatinine 5, sedimentation rate >100, HIV negative, p-ANCA positive and c-ANCA negative. Serum and urine protein electrophoresis were normal. Renal biopsy demonstrated p-ANCA associated crescentic glomerulonephritis without evidence of amyloid. Workup for systemic amyloidosis including bone marrow aspirate, fat pad aspirate and cardiac MRI was negative.
DISCUSSION: Tracheobronchial amyloidosis demonstrates diffuse submucosal deposition of amyloid plaques and nodules along the airways with varying degrees of obstruction 1. It presents with recurrent pneumonia, respiratory failure and may mimic bronchogenic carcinoma. Management includes observation, intermittent bronchoscopic resection or laser therapy 2,3.
CONCLUSIONS: Tracheobronchial amyloidosis should be considered in differential diagnosis of recurrent pneumonia. Bronchoscopy is required for establishing diagnosis by tissue biopsy and for treatment.
Reference #1: O’Regan A et al .Tracheobronchial amyloidosis: the Boston University experience from 1984 to 1999. Medicine 2000; 79, 69-79
Reference #2: Hui AN et al. Amyloidosis presenting in the lower respiratory tract. Arch Pathol Lab Med. 1986;110, 212-8
Reference #3: Cordier JF et al. Amyloidosis of the lower respiratory tract. Clinical and pathologic features in a series of 21 patients. Chest. 1986; 90, 827-31
DISCLOSURE: The following authors have nothing to disclose: Elvina Khusainova, Mohit Chawla
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