Systemic amyloidoses are rare, complex diseases caused by misfolding of autologous protein. Although these diseases are fatal, effective treatments exist that can alter their natural history, provided that they are started before irreversible organ damage has occurred. The cornerstones of the management of systemic amyloidoses are early diagnosis, accurate typing, appropriate risk-adapted therapy, tight follow-up, and effective supportive treatment. Internists play a key role in suspecting the disease, thus allowing early diagnosis, starting the diagnostic workup and selecting patients that should be referred to specialized centers, judiciously titrating supportive measures, and following patients throughout the course of the disease. Here we review the pathogenesis, diagnosis and treatment of the most common forms of systemic amyloidoses.
Systemic amyloidoses: what an Internist should know
PALLADINI, GIOVANNI;MERLINI, GIAMPAOLO
2013-01-01
Abstract
Systemic amyloidoses are rare, complex diseases caused by misfolding of autologous protein. Although these diseases are fatal, effective treatments exist that can alter their natural history, provided that they are started before irreversible organ damage has occurred. The cornerstones of the management of systemic amyloidoses are early diagnosis, accurate typing, appropriate risk-adapted therapy, tight follow-up, and effective supportive treatment. Internists play a key role in suspecting the disease, thus allowing early diagnosis, starting the diagnostic workup and selecting patients that should be referred to specialized centers, judiciously titrating supportive measures, and following patients throughout the course of the disease. Here we review the pathogenesis, diagnosis and treatment of the most common forms of systemic amyloidoses.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
