DYSPNEA IN THE CONGO LINE

Abstract

SESSION TITLE: Lung Pathology 1 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Amyloidosis is a spectrum of diseases characterized by the extracellular infiltration of fibrillar proteins into various tissues of the body. Organs typically involved include the heart and kidneys, but other organs can be affected as well. Amyloidosis isolated to the lungs is reported rarely in the literature. CASE PRESENTATION: We present a case of a 45-year-old male who presented to the pulmonary clinic for an abnormal CT scan of his chest. His medical history included latent tuberculosis, Sjogren’s syndrome, testicular cancer, and chronic kidney disease with a renal biopsy showing mesangial IgM deposition. He reported a 30-pack year smoking history. His exam was remarkable only for poor dentition. CT of his chest revealed multiple cysts and nodules, and his pulmonary function tests showed only borderline air trapping. He was counseled on smoking cessation. Six months later, he began to experience dyspnea and fatigue, and PFTs revealed a new mild obstructive pattern. Repeat chest CT demonstrated enlarging nodules and ground glass opacities. PET CT showed avidity of a cystic lesion in the left lung as well as asymmetry in parotid gland avidity. Bronchoscopy discovered diffuse mucosal friability and endobronchial lesions at the right middle lobe takeoff as well as the left lower lobe. Endobronchial biopsy results described amorphous material with Congo red staining consistent with amyloidosis. Bone marrow biopsy, echocardiogram, and fat pad biopsy were all negative for evidence of systemic disease. Parotid gland biopsy revealed marginal zone lymphoma. Treatment with bedamustine and rituximab resulted in symptom and imaging stabilization. DISCUSSION: Amyloidosis is characterized by apple-green birefringence under polarized light after Congo red staining. Disease can be localized or systemic, hereditary or acquired, and primary or secondary. Amyloidosis affecting the lung is usually associated with systemic disease, but localized forms can also occur. These localized forms tend to be of the tracheobronchial subtype as opposed to nodular or alveolar-septal types and composed of AL fibril proteins. Most case reports of tracheobronchial amyloidosis in the literature describe middle-aged patients with a slight predilection for males. Symptoms generally include dyspnea, cough, wheezing, and hemoptysis. CT scan of the chest is important for identifying abnormalities but endobronchial tissue sampling is necessary for diagnosis. Interestingly, nodular AL pulmonary amyloidosis, which is generally associated with systemic disease, has been associated with MALT lymphoma. Localized tracheobronchial amyloidosis in the setting of MALT lymphoma appears to be less frequently described but has been associated with Sjogren’s disease. CONCLUSIONS: Recognition of atypical presentations of amyloidosis can lead to earlier interventions and diagnosis of associated systemic disease. Reference #1: Khoor, A., & Colby, T. V. (2017). Amyloidosis of the Lung. Archives of Pathology & Laboratory Medicine, 141(2), 247–254. https://doi.org/10.5858/arpa.2016-0102-RA DISCLOSURES: No relevant relationships by Audrey Haywood, source=Web Response