Abstract
Systemic amyloidoses are rare protein deposition disorders, which are often diagnosed in an advanced stage of the disease due to non-specific symptoms. Any chronic inflammatory disease can lead to an AA-type amyloidosis. This paper summarizes the current state of the art of diagnosis and treatment of AA amyloidosis and presents data from the past 10years of our amyloidosis center. Our data represents an analysis of our cohort of patients with amyloidosis and aselective research in the PubMed database for AA amyloidosis. The underlying diseases comprise autoinflammatory syndromes, polyarthritis, and chronic inflammatory bowel and lung diseases. Renal organ involvement is the most prevalent in AA amyloidosis. It can be detected early through the evaluation of proteinuria. The treatment depends on the individual underlying disease. Patients without an associated inflammatory disease are considered to have idiopathic AA amyloidosis and empiric treatment is mandatory. Survival of this fatal disease has recently improved due to the new diagnostic tools and treatment options; however, early diagnosis plays acrucial role in the prevention of end-stage renal failure. New therapeutic strategies aim to remove existing amyloid deposits.
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