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• W2894746989 abstract "SESSION TITLE: Pulmonary Manifestations of Systemic Disease 1 SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/09/2018 03:45 PM - 04:45 PM INTRODUCTION: Amyloidosis is an uncommon disease which usually affects multiple organs. Isolated pulmonary amyloidosis is even rarer, mainly manifesting as nodular or Tracheobronchial Amyloidosis (TBA). We present a rare case of isolated secondary (AA) amyloidosis of the tracheobronchial tree. CASE PRESENTATION: 53 year old female was referred to Pulmonology clinic for evaluation of chronic cough and recurrent episodes of bronchitis. She had 63 pack year smoking history and subsequently had developed COPD. Examination showed coarse breath sounds bilaterally with normal oxygen saturation. Computed Tomography (CT) chest showed peribronchial wall thickening, right middle lobe nodular infiltrate with bronchiectasis and calcified nodules in the right lower lobe. Outpatient flexible bronchoscopy revealed lumpy, edematous, erythematous and highly friable endobronchial mucosa, more on the right lung. The procedure was complicated with significant bleeding necessitating prompt intubation and admission to the unit. Endobronchial biopsy showed homogenous material with morphologic, Congo red and immunoperoxidase features consistent with amyloid deposition; it was positive for Amyloid P and negative for Amyloid A proteins. Culture was positive for Pseudomonas. Extensive work up for plasma cell dyscrasias, including fat pad biopsy, as cause of her amyloidosis was negative. Once she was extubated safely and treated for pneumonia, she underwent endoscopic debulking. Multidisciplinary committee recommended maximal treatment of her COPD and optimal pulmonary hygiene with the aims of improving symptoms, reducing secretions and preventing infections. She had only one uncomplicated episode of Pneumonia in the next 6 months. DISCUSSION: Pulmonary amyloidosis may be endobronchial, interstitial, nodular or diffuse parenchymal. Endobronchial type is one of the rarest diseases and presents most often with obstructive symptoms. Bronchoscopy with endobronchial biopsy is diagnostic for TBA. Given the rarity of these cases, most effective treatment is not known. Management options include conservative therapy, external beam radiation therapy, and endobronchial or surgical excision. Surgery is reserved for complicated cases. Subtyping of amyloid protein is necessary to determine the need for radiation therapy. Conservative therapy is aimed at maximizing the treatment of co-existing lung diseases and maintaining clear airways by minimizing daily sputum production and optimizing clearance. CONCLUSIONS: Clinicians should recognize pulmonary tracheobronchial amyloidosis as a rare cause of obstructive lung disease. It is extremely important to differentiate light chain (AL) amyloidosis from secondary (AA) amyloidosis as the cause of TBA as the treatment options for the two are widely different, with the latter focused on conservative measures. Reference #1: Haiqing Chu, Lan Zhao, et. al: Clinical characteristics of amyloidosis with isolated respiratory system involvement: A review of 13 cases. Annals of Thoracic Medicine. 2013; 7:243-49 Reference #2: Hoskote, Sumedh et al. Tracheobronchial Amyloidosis: Therapy and Outcomes in 24 Patients. CHEST , Volume 148 , Issue 4 , 798A Reference #3: Ding, L., Li, W., Wang, K., Chen, Y., Xu, H., Wang, H.. (2010) Primary tracheobronchial amyloidosis in China: analysis of 64 cases and a review of literature. J Huazhong Univ Sci Technolog Med Sci 30: 599–603. DISCLOSURES: No relevant relationships by Alexander Goldstein, source=Web Response No relevant relationships by Muhammad Habib, source=Web Response No relevant relationships by Muhammad Malik, source=Web Response No relevant relationships by Manish Patel, source=Web Response No relevant relationships by Grace Wang, source=Web Response No relevant relationships by Kishore Yalamanchili, source=Web Response" @default.
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• W2894746989 date "2018-10-01" @default.
• W2894746989 modified "2023-09-27" @default.
• W2894746989 title "TRACHEOBRONCHIAL AMYLOIDOSIS MIMICKING CHRONIC BRONCHITIS" @default.
• W2894746989 doi "https://doi.org/10.1016/j.chest.2018.08.833" @default.
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