The term " @entity2783 " encompasses the heterogeneous group of diseases caused by the extracellular deposition of autologous fibrillar proteins. The global incidence of @entity2783 is estimated at five to nine cases per million @entity1 -years. While @entity3917 ( @entity3917 ) @entity2783 is more frequent in developed countries, @entity1129 ( @entity1129 ) @entity2783 is more common in some European regions and in developing countries. The spectrum of @entity2783 has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary @entity2783 with respect to the @entity1129 type; and a substantial change in the epidemiology of the underlying diseases. Diagnosis of @entity2783 is based on clinical organ involvement and histological evidence of @entity527 . Among the many tinctorial characteristics of @entity527 , avidity for Congo red and metachromatic birefringence under unidirectional polarized light remain the gold standard. Once the initial diagnosis has been made, the amyloid subtype must be identified and systemic organ involvement evaluated. In this sense, the (123)I-labeled serum amyloid P component scintigraphy is a safe and noninvasive technique that has revolutionized the diagnosis and monitoring of treatment in @entity1582 . It can successfully identify anatomical patterns of @entity527 throughout the body and enables not only an initial estimation of prognosis, but also the monitoring of the course of the disease and the response to treatment. Given the etiologic diversity of @entity2783 , common therapeutic strategies are scarce. All treatment options should be based upon a greater control of the underlying disease, adequate organ support, and treatment of symptoms. Nevertheless, novel therapeutic strategies targeting the formation of amyloid fibrils and @entity527 may generate new expectations for @entity1 with @entity2783 .

XXXX : epidemiology, diagnosis, and management.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity527 :: ('MESH:D058225', 'Disease') :: ['amyloid deposits', 'amyloid deposition'] @entity2783 :: ('MESH:D000686', 'Disease') :: ['AA amyloidosis', 'amyloidosis', 'AL amyloidosis'] @entity1582 :: ('MESH:D009101', 'Disease') :: ['systemic amyloidosis'] @entity1129 :: ('MESH:D001260', 'Disease') :: ['AA', 'amyloid A'] @entity3917 :: ('MESH:D020795', 'Disease') :: ['amyloid light-chain', 'AL']
@entity1582 :: (MESH:D009101,Disease) :: ['Systemic AA amyloidosis']
