대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Amyloidosis

Radiologic Findings

Chest radiograph shows a nodular lesion with well defined inferior margin in LUL. Contrast enhanced axial chest CT scan showed 3.0cm sized well defined, oval shaped, pleura based mass in LUL. After 22 months, newly seen several subpleural nodules were observed.

Brief Review

Amyloidosis is a heterogenous group of disorders associated with extracellular deposition of protein in various organs in an abnormal fibrillar form. Amyloidosis may present in systemic or localised forms, and pulmonary involvement may be localized, as in localized pulmonary amyloidosis, or represent part of a systemic process (systemic amyloidosis). Systemic amyloidosis can be further classified into primary and secondary systemic amyloidosis. Primary systemic amyloidosis (AL) is associated with an underlying monoclonal dyscrasia of plasma or B-lymphoid cells. In patients with primary amyloidosis, nearly one-third are ultimately diagnosed with either multiple myeloma or B-cell lymphoma, whereas approximatedly 10% to 15% of patients with myeloma or lymphoma develop amyloidosis. Secondary systemic amyloidosis (AA) is associated with chronic conditions such as tuberculosis, chronic renal disease, rheumatoid arthritis and ankylosing spondylitis.
Pulmonary involvement in systemic amyloidosis is not uncommon, four distinct patterns of thoracic involvement of may be identified: (a) trachobronchial disease with airway wall thickening, nodular deposits of amyloid; (b) nodular parenchymal disease (c) diffuse alveolar septal disease (d) nodal disease. Nodular pulmonary amyloidosis (seen in the present case) carries the most favorable prognosis and long-term survival is expected even without treatment. Patients are typically asymptomatic and incidental findings of pulmonary nodules on routine chest radiographs are a common mode of presentation, although some may present with cough, shortness of breath and hemoptysis. However, a solitary nodule is a more common finding than multiple nodules on chest radiograph. On CT, pulmonary nodules have sharp and lobulated margins, and are usually found in a subpleural or peripheral location, more frequently in the lower lobes. The size of the nodules may range from micronodular to 15 cm in diameter. Over time, nodules may grow slowly and cavitate, calcify, or resolve spontaneously. Calcification can be seen in 20%–50% of nodules on CT images.

References

1. Webb, W. Richard, Nestor L. Muller, and David P. Naidich. High-Resolution CT of the Lung. Lippincott Williams & Wilkins, 2014;419-423
2. Aylwin, Anthony C. B., Philip Gishen, and Susan J. Copley. “Imaging Appearance of Thoracic Amyloidosis.” Journal of Thoracic Imaging 2005;1: 41–46.
3. Chew, Kian Ming, Michael John Clarke, Niraj Dubey, and Ju Ee Seet. “Nodular Pulmonary Amyloidosis with Unusual, Widespread Lung Cysts.” Singapore Medical Journal 54;5: e97–99.

Keywords

pleura, Metabolic and storage lung disesae,