Causes of AA amyloidosis: a systematic review

Abstract

From a clinical perspective, there is a need for a reliable and comprehensive list of diseases causing AA amyloidosis. This list could guide clinicians in the evaluation of patients with AA amyloidosis in whom an obvious cause is lacking. In this systematic review, a PubMed, Embase and Web of Science literature search were performed on causes of AA amyloidosis published in the last four decades. Initially, 4066 unique titles were identified, but only 795 full-text articles and letters were finally selected for analysis. Titles were excluded because of non-AA type of amyloidosis, language, no fulltext publication or irrelevance. Hundred and fifty diseases were initially reported to be associated with the development of AA amyloidosis. The presence of AA amyloid was proven in 208 articles (26% of all) of which 140 (67%) showed a strong association with an underlying disease process. Disease associations were categorized and 48 were listed as strong, 19 as weak, 23 as unclear, and 60 as unlikely. Most newly described diseases are not really unexpected because they often cause longstanding inflammation. Based on the spectrum of identified causes, a pragmatic diagnostic approach is proposed for the AA amyloidosis patient in whom an obvious underlying disease is lacking.

Amyloidosis and Renal Disease in Patients with Crohn's Disease

“…), and chronic inflammatory diseases (mainly rheumatic but inflammatory bowel disease [IBD] as well). Amyloidosis is a rare but serious complication of IBD, being more frequent in patients with CD than in those with ulcerative colitis (UC) [8].…”

Renal AA amyloidosis leading to early diagnosis and treatment of takayasu arteritis: a case report and review of the literature

“…The involvement can vary from mild proteinuria to renal failure or nephrotic syndrome. While in developing countries, the most common cause of AA amyloidosis is infections, in western countries, it is commonly associated with autoimmune diseases [ 8 ]. Rheumatoid arthritis, juvenile idiopathic arthritis and ankylosing spondylitis are the most frequent underlying diseases associated with AA amyloidosis, accounting for 23–51%, 7–48% and 0–12% of all cases, respectively [ 9 ].…”

“…The association between TA and AA amyloidosis has been described, but due to the low incidence of both conditions, only a few cases are reported in the literature [ 8 ]. Table 1 A shows the most common causes of AA amyloidosis, while Table 1 B shows the prevalence of TA in different countries.…”

“… * This table reflects data of several epidemiological studies with different methods and diagnostic criteria [ 8 ] …”

“…Few reports have demonstrated an association between AA amyloidosis and Takayasu arteritis [ 8 , 11 , 12 ], and only in a few cases was AA amyloidosis the initial manifestation of TA [ 11 , 12 ]. Considering the slow progression and sometimes long periods of subclinical disease observed in TA, it is reasonable to suggest that AA amyloidosis, in this scenario, shows a different natural history when compared to other diseases, such as rheumatoid arthritis, in which the clinical manifestations and diagnosis are present for many years before the onset of AA amyloidosis.…”