A rare case of nodular pulmonary amyloidosis associated with sjogren's syndrome

SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 6 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 12:00 pm - 12:45 pm INTRODUCTION: Pulmonary amyloidosis can appear in the lung in three forms: nodular pulmonary amyloidosis (NPA), diffuse alveolar-septal amyloidosis, and tracheobronchial amyloidosis. NPA is caused by abnormal protein deposition, with Sjogren's syndrome being a rare cause. Our case describes a patient with an incidental finding of pulmonary nodules, which led to the diagnosis of NPA with Sjogren's syndrome. CASE PRESENTATION: A 68-year-old woman with history of hypothyroidism and no smoking history presented for evaluation of incidentally found pulmonary nodules in the chest X-ray. The patient was asymptomatic except for a long-standing complaint of dry eyes. The chest X-ray and chest CT revealed multiple bilateral diffuse pulmonary nodules with calcification measuring up to 2.7cm. PET-CT scan revealed numerous bilateral pulmonary nodules of variable sizes, with maximum SUV of 4.6. There was no other organ system amyloidosis involvement. The nodule with the highest SUV was biopsied and liquid chromatography-tandem mass spectrometry was performed on peptides extracted from Congo red-positive red staining tissue and was consistent with immunoglobulin-associated amyloid. Other autoimmune testing revealed high antinuclear antibody (ANA) levels of 12U and an elevated anti-SSA antibody level of 8 U, leading to a Sjogren syndrome diagnosis. Immunoglobulin testing showed high kappa (7.02 mg/dl) and lambda-free light chain (5.12mg/dl) levels. As the patient was asymptomatic, regular monitoring of her amyloid lung nodules and symptoms was chosen as the treatment approach. DISCUSSION: NPA is a form of pulmonary amyloidosis that is usually localized and incidentally found on chest imaging [1, 2]. It usually represents localized immunoglobulin light chain (AL) amyloidosis, and most cases are the result of an underlying lymphoproliferative disorder such as MALT lymphoma and Sjogren's syndrome [1]. Most cases of NPA are usually asymptomatic, and the most common radiographic abnormalities are nodules with or without calcifications. However, chest imaging has a poor ability to differentiate amyloidosis from other diseases. Therefore, diagnosing NPA relies on a thorough evaluation, including symptoms, image studies, electrophoresis, autoimmune profile, and biopsy. Most patients with NPA can be managed conservatively with a good long-term prognosis . CONCLUSIONS: The occurrence of Nodular pulmonary amyloidosis (NPA) is rare. However, it should be included in the differential diagnosis of pulmonary nodules especially in patients with lymphoproliferative disorders. REFERENCE #1: Milani P, Basset M, Russo F, Foli A, Palladini G, Merlini G. The lung in amyloidosis. Eur Respir Rev. 2017 Sep 6;26(145):170046. doi: 10.1183/16000617.0046-2017 REFERENCE #2: Kinoshita Y, Ikeda T, Miyamura T, Ueda Y, Yoshida Y, Ueda M, Haraoka S, Kushima H, Ishii H. Nodular Pulmonary Amyloidosis Associated with Sjögren's Syndrome. Intern Med. 2022 Mar 15;61(6):877-881. doi: 10.2169/internalmedicine.8169-21. DISCLOSURES: No relevant relationships by Scott Helgeson No relevant relationships by Sadhana Jonna No relevant relationships by Angela K No relevant relationships by Jorge Sinclair De Frías No relevant relationships by Kate Walsh