CT evaluation of amyloidosis: spectrum of disease
Amyloidosis is a rare systemic disease caused by extracellular deposition of an insoluble protein. Although it is usually seen in a systemic form, 10%-20% of cases can be localized. Systemic amyloidosis is subclassified into an idiopathic primary form and a secondary or reactive form. Patients with...
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Veröffentlicht in: | Radiographics 1993-11, Vol.13 (6), p.1295-1308 |
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Zusammenfassung: | Amyloidosis is a rare systemic disease caused by extracellular deposition of an insoluble protein. Although it is usually
seen in a systemic form, 10%-20% of cases can be localized. Systemic amyloidosis is subclassified into an idiopathic primary
form and a secondary or reactive form. Patients with primary amyloidosis have no underlying condition or disease. Men are
affected more than women, and the mean age at presentation is 55-60 years. Some causes of secondary amyloidosis are multiple
myeloma (10%-15%), rheumatoid arthritis (20%-25%), tuberculosis (50%), or familial Mediterranean fever (26%-40%). Radiographic
studies of 90 patients with biopsy-proved primary or secondary amyloidosis were reviewed. Computed tomographic (CT) scans
demonstrated a wide spectrum of disease in the cardiothoracic, gastrointestinal, genitourinary, and musculoskeletal systems.
Amyloid deposition simulated both inflammatory and neoplastic conditions. Amorphous or irregular calcifications were occasionally
identified within the amyloid deposit. Definitive diagnosis requires biopsy confirmation, as CT findings are nonspecific. |
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ISSN: | 0271-5333 1527-1323 |
DOI: | 10.1148/radiographics.13.6.8290725 |