Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Amyloidosis
- Radiologic Findings
- Fig 1. Chest PA shows multiple nodules in both lungs.
Fig 2-5. CT scan reveals multiple and variable size lung nodules with calcification and variable size cystic lesions in both lungs.
- Brief Review
- Amyloidosis is a group of diseases resulting from the extracellular accumulation of abnormal protein in tissues and organs throughout the body. It exists as a primary disease or, more commonly, may be secondary to a wide variety of pathologic processes ranging from chronic infection or inflammation to malignancy. Patients with SLE are rarely associated with pulmonary amyloidosis (usually AA type).
Pulmonary amyloidosis is more often a localized process instead of a part of systemic disease. Two patterns of involvement exist: the nodular parenchymal and the alveolar septal forms. Radiologic findings in both subtypes are often nonspecific.
On chest CT, nodular parenchymal amyloidosis may manifest as solitary or multiple pulmonary nodules and may mimic a variety of diseases from granulomatous disease to malignancy. Multiple pulmonary nodules are common and may exhibit smooth, lobulated, or speculated margins and occasional central or punctate calcification. Rarely, lesions may cavitate. The natural history of nodular parenchymal amyloidosis is benign. Lesions may slowly grow in size and number, but the overall prognosis is excellent and treatment is rarely required.
Alveolar septal pulmonary amyloidosis is characterized by well-defined 2-4mm micronodules accompanied by reticular opacities, interlobar septal thickening, and confluent consolidations with basal and peripheral predominance. Punctate lung calcifications, pleural effusions, and pleural thickening also may occur. Lung cysts have rarely been described, often in patients with systemic amyloidosis due to Sjogren syndrome. The cysts may be associated with calcified or noncalcified soft-tissue nodules. Whether the cysts are a manifestation of lymphocytic interstitial pneumonia in the setting of Sjogren syndrome or a manifestation of amyloidosis is unclear. Compared with nodular pulmonary amyloidosis, patients with diffuse alveolar septal amyloidosis have a far worse prognosis with a median survival of 16 months.
- Please refer to
Case 1203, Case 1097, Case 1038, Case 998, Case 985, Case 982, Case 939, Case 927, Case 900, Case 834, Case 794, Case 786, Case 738, Case 664, -
KSTR imaging conference 2017 Summer Case 10
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KSTR Imaging Conference 2015 Spring Case 4
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KSTR Imaging Conference 2011 Spring Case 8,
- References
- 1. Czeyda-Pommersheim F, Hwang M, Chen SS, Strollo D, Fuhrman C, Bhalla S. Amyloidosis: modern cross-sectional imaging. Radiographics 2015;35(5):1381-1392.
2. Ellender CM, McLean C, Williams TJ, Snell GI, Whitford HM. Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension. Respirology Case Reports 2015;3(2):78-81.
- Keywords