AmyloidosisAmyloidosis—Recent Developments
Section snippets
Methods
PubMed and MEDLINE databases (1990-2007) and internet were searched for the keyword “amyloidosis” (or occasionally closely related words, such as amyloid or amyloid deposit) in articles published in English. This search strategy revealed 7864 index abstracts. Some of the references were found with special keywords: 13 using “nephrotic syndrome,” 16 using “natriuretic peptide,” 31 using “periodic fever,” and 50 using “Muckle–Wells syndrome.” In particular, more recent articles pertinent to this
Clinical Suspicion of Amyloidosis
Certain symptoms and signs, which can be referred to as “amyloid syndromes,” in the context of chronic infectious/inflammatory diseases, plasma cell dyscrasias, or family history, arouse a clinical suspicion of amyloidosis. In particular, proteinuria, nephrotic syndrome, or renal failure is suspicious in this respect. Enteropathy leading to generalized malabsorption or gastrointestinal bleeding is suggestive of AA amyloidosis, whereas restrictive cardiomyopathy with thromboemboli and
Discussion
The keystone in the diagnosis of amyloidosis is clinical suspicion, which is not easily raised, because despite their shared molecular pathomechanisms the “amyloid syndromes” are heterogenous in their clinical expression. In contrast to many other diseases, the diagnosis of amyloidosis cannot be based on a serum sample but requires histopathologic demonstration of tissue deposition of amyloid using Congo red and type-specific antibodies. As 27 different subtypes have been described, specific
Acknowledgments
This study was supported by Finska Läkaresällskapet and the Else and Wilhelm Stockmann Foundation.
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