THE RARE ENTITY OF PULMONARY AMYLOIDOSIS

Abstract

SESSION TITLE: Diffuse Lung Disease 2 SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/07/2018 04:45 pm - 05:45 pm INTRODUCTION: Pulmonary amyloidosis is an uncommon disease, characterized by extracellular deposition of fibrillary protein in the lungs. It appears in three forms: tracheobronchial, nodular pulmonary, and alveolar septal. We report a rare case of single, nodular pulmonary amyloidosis in a middle aged Hispanic patient. CASE PRESENTATION: A 43 year old Hispanic female, never smoker, was referred to our Chest clinic by her gastroenterologist, when an abdominal imaging for possible gallstones revealed a 2.1cm speculated right middle lobe nodule. A dedicated chest Ct then confirmed the nodule but also revealed several thin walled lung cysts of various sizes. She denied any pulmonary or constitutional symptoms other than occasional dry cough. Her Pulmonary function test was normal. Patient then underwent CT guided biopsy of the lesion for concern of malignancy. Histopathologic examination of the biopsy specimen revealed it to be nodular parenchymal amyloidosis of localized AL type. Full work up for amyloidosis then revealed possible renal involvement. Patient currently being followed up by nephrology and us for further management DISCUSSION: Amyloidosis is characterized by extra-cellular deposits of an insoluble fibrillar protein (amyloid) with a corrugated plate structure. Upon reacting with Congo red, the protein exhibits an apple-green birefringence under a polarized microscope. The mean age of onset is 55-60 years. Cases with kappa and lambda light chain deposits are referred to as amyloid light chain (AL) amyloidosis, and those with plasma amyloid P deposits due to chronic infectious disease are called amyloid A (AA) amyloidosis. Amyloidosis localized to the respiratory system is very rare, and is classified as parenchymal nodular, tracheobronchial, or diffuse alveolar septal according to the type of pulmonary invasion involved. Cysts are extremely rare. The tracheobronchial type can accompany a variety of symptoms, including dyspnea, hemoptysis, and cough, but it can also be asymptomatic in .Pulmonary parenchymal nodular type is mostly asymptomatic. The diffuse alveolar septal type exhibits progressive pulmonary fibrosis patterns, and most patients experience respiratory failure leading to death. Management of localized pulmonary amyloidosis is dependent on the severity of symptoms, systemic or localised and the type of amyloid protein. Asymptomatic patients may not require treatment. CONCLUSIONS: A single nodular lesion can mimic various conditions, including malignancy, pulmonary tuberculosis, and fungal infection. Once single nodular lesions are observed, it is necessary to have a broad differential diagnosis including amyloidosis.Our case is unique in its rarity and atypical presentation of pulmonary amyloid and also its occurrence in a younger population than described in literature. Reference #1: Lee SH, Ko YC, Jeong JP, et al. Single Nodular Pulmonary Amyloidosis: Case Report. Tuberculosis and Respiratory Diseases. 2015;78(4):385-389. https://doi.org/10.4046/trd.2015.78.4.385. Reference #2: Andras Khoor and Thomas V. Colby (2017) Amyloidosis of the Lung. Archives of Pathology & Laboratory Medicine: February 2017, Vol. 141, No. 2, pp. 247-254. DISCLOSURES: No relevant relationships by Prarthna Chandar, source=Web Response No relevant relationships by Sakthidev Kulandaisamy, source=Web Response No relevant relationships by Ben Shamian, source=Web Response No relevant relationships by Shyam Shankar, source=Web Response no disclosure on file for Jack Twersky