PRIMARY PULMONARY NODULAR AMYLOIDOSIS IN A FEMALE WITH UNDIAGNOSED SJOGREN'S SYNDROME

Abstract

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Pulmonary amyloidosis is characterized by extracellular deposition of insoluble, misfolded fibrils within the lung. Isolated pulmonary amyloidosis is a rare condition especially in association with Sjogren’s syndrome. Frequently asymptomatic, we report a case of nodular pulmonary amyloidosis in a young, Hispanic female who presented with dyspnea. CASE PRESENTATION: A 45 year-old female presented with shortness of breath and chest tightness for 3 years. Her symptoms progressed in frequency, functional status deteriorated and she experienced a 20-pound weight loss in the past year. Initial imaging 2 years prior revealed multiple lung nodules with a thin-walled cyst in the right middle lobe (RML). Quantiferon-TB Gold and coccidioidomycosis serology was negative. Pulmonary function testing (PFT) demonstrated a minimal obstructive lung defect with mild response to bronchodilator. Repeat imaging demonstrating increasing size of multiple pulmonary nodules, a now solid nodule of RML, and a 2.6 cm left suprasternal lymph node. Computed tomography (CT)-guided biopsy revealed nodular pulmonary amyloidosis confirmed with congo red stain. The patient was placed on a prednisone taper with improvement of symptoms. She continued to endorse multiple recurring subcutaneous nodules of both upper and lower extremities, dry mouth, dry eyes, cold fingers, and hair loss. Lab studies were notable for positive anti-SSA/Ro antibodies and rheumatoid factor (208 IU/mL), and negative cyclic citrullinated peptide antibody and antinuclear antibody. Serum protein electrophoresis identified a mild elevation of gamma globulins. Plaquenil, Rituximab and Methotrexate therapy was initiated. Repeat PFT had normal spirometry, lung volume and diffusion capacity. DISCUSSION: Pulmonary amyloidosis appears in three forms: pulmonary nodules, tracheobronchial, and diffuse alveolar septal. Nodular pulmonary amyloidosis is often an incidental finding and is indistinguishable from neoplasm or granulomatous lesions. Nodules are slowly enlarging and may cavitate or calcify. Histological examination is necessary to determine the diagnosis. In this case, untreated Sjögren’s syndrome with gammaglobinemia likely led to deposition of amyloid in lung. In literature, PFT are mostly normal with a small subset demonstrating restrictive pattern and even fewer with reduced diffusion. Our patient initially presented with obstructive pattern likely due to the large cyst compromising elastic recoil of the lungs. CONCLUSIONS: In primary nodular pulmonary amyloidosis, nodules are radiographically nonspecific and require biopsy for diagnosis. Amyloidosis should be included in the differential diagnosis of multiple pulmomary nodules. Management is not well defined due to the low incidence of the disease. When associated with Sjögren’s syndrome, there is reported success with treatment of steroids and immunosuppression. Reference #1: Xian, Jonathan Z. et all. "A 45-Year-Old Woman With Multiple Pulmonary Nodules and Sjogren Syndrome.” CHEST 2019;155(2):e51-154. Reference #2: Heraganahally, Subash et all. "Pulmonary AL - amyloidosis masquerading as lung malignancy in an Australian Indigenous patient with Sjogren's syndrome.” Respiratory Medicine Case Reports 2019;26:94-97 Reference #3: Garcia-Sanchez, Aldara et all. "Amyloidosis as a Cause of Cystic Pulmonary Fibrosis Associated With Pulmonary Nodules.” Arch Bronconeumol 2018;54(9): 481-494. DISCLOSURES: No relevant relationships by Aarti Mittal, source=Web Response No relevant relationships by Erin Wiltchik, source=Web Response