A Unique Case of Systemic Amyloidosis Presenting With Recurrent Pleural Effusion

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 3 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Systemic amyloidosis can affect multiple organ systems. Pulmonary amyloidosis most commonly affects the lung parenchyma but can affect any part of the respiratory tract. Persistent pleural effusion develops in 1 to 2 percent of patients and is a poor marker of outcome. A diagnostic challenge exists to distinguish primary effusions from effusion due to cardiomyopathy. We hereby present a case with persistent pleural effusion refractory to diuretics and multiple hospital admissions. CASE PRESENTATION: Our patient is a 75-year-old African American male, who presented with worsening dyspnea for 1 year, found to have recurrent pleural effusion that warranted multiple hospital admissions and thoracentesis and was admitted to our hospital with the same complaints. Review of records showed monoclonal bands on SPEP and increased lambda chains; Cardiac MRI showed diffuse subendocardial late gadolinium enhancement and Echo showed restrictive LV filling pattern. Thoracentesis was consistent with a transudative effusion and negative for malignancy. Right and Left heart catheterization showed patent coronaries, RVEDP/RVSP ratio <0.3, indicative of restrictive cardiomyopathy. Abdominal fat pad biopsy was positive for Congo red stain with polarization. Bone marrow biopsy showed a plasma cell disorder being managed by oncology. DISCUSSION: Amyloidosis is a rare disorder incidence of AL amylodosis reported to be about 6 to 10 cases per million person years. It involves the formation of abnormal protein fibrils and deposition of these fibrils within various organs and tissues throughout the body. Pulmonary involvement with persistent pleural effusions is rare and postulated to be due to amyloid-induced cardiomyopathy, nephrotic syndrome and impaired fluid resorption. Our patient met all four Diagnostic criteria established by the Mayo Clinic and International Myeloma Working Group for amyloidosis and had a unique presentation of recurrent pleural effusion. Current treatment guidelines for AL amyloidosis involve the use of chemotherapy and autologous stem cell transplantation. CONCLUSIONS: Our patient’s presentation interesting because Systemic Amyloidosis is a rare condition and persistent pleural effusion as a manifestation of amyloidosis is even rarer occurring in 1-2 percent of cases of systemic amyloidosis. Because they portend a poor prognosis with a poor survival, prompt diagnosis is critical in order to initiate treatment. Reference #1: Alappan N., Dobkin J., Klapper P. A rare cause of pleural effusion. American Journal of Respiratory and Critical Care Medicine. 2012;185, article A5191 Reference #2: Seth H. G. M., Desai S., Sein V., Shinar B., Mathews M. Persistent pleural effusion in systemic AL amyloidosis. American Journal of Respiratory and Critical Care Medicine. 2013;187A6132 Reference #3: Tada L, Anjum H, Linville WK, Surani S. Recurrent Pleural Effusions Occurring in Association with Primary Pulmonary Amyloidosis. Case Reports in Pulmonology. 2015;2015:421201. doi:10.1155/2015/421201. DISCLOSURE: The following authors have nothing to disclose: Osman Ahmedtaha, Shahabuddin Soherwardi, Alem Mehari, Jennifer Obi No Product/Research Disclosure Information