Secondary Amyloidosis
Özet
Secondary (AA) amyloidosis is a systemic disorder resulting from the accumulation of serum amyloid A (SAA) protein, typically in the setting of chronic inflammation. Although primarily affecting the kidneys, cardiac involvement—though rare—can occur and is associated with poor prognosis. AA amyloidosis is often linked to chronic infections, autoimmune diseases, and hereditary autoinflammatory conditions like familial Mediterranean fever (FMF). Diagnosis relies on histological confirmation, but imaging techniques such as echocardiography and cardiac magnetic resonance (CMR) imaging play an essential role, particularly in evaluating cardiac involvement. Treatment strategies aim to control the underlying inflammatory condition and reduce SAA levels. Traditional therapies include colchicine, non-steroidal anti-inflammatory drugs, and glucocorticoids, while newer targeted therapies such as anti-TNF, anti-IL-1, and anti-IL-6 agents have shown promise. Renal involvement may necessitate supportive care, dialysis, or kidney transplantation. However, patients with cardiac involvement exhibit significantly higher mortality, especially post-transplantation. Novel imaging techniques and targeted biologics continue to enhance disease management, although complete resolution of amyloid deposits remains elusive. Early recognition and treatment of the underlying cause are crucial for improving outcomes. Despite advances, AA amyloidosis remains a challenging condition requiring multidisciplinary management.
Sekonder (AA) amiloidoz, genellikle kronik inflamasyon varlığında serum amiloid A (SAA) proteinlerinin birikimiyle ortaya çıkan sistemik bir hastalıktır. En sık böbrekleri etkileyen bu hastalıkta, nadiren de olsa kalp tutulumu görülebilir ve bu durum prognozu belirgin şekilde kötüleştirir. AA amiloidoz; kronik enfeksiyonlar, otoimmün hastalıklar ve Ailevi Akdeniz Ateşi (FMF) gibi kalıtsal otoinflamatuar sendromlarla ilişkilidir. Tanı çoğunlukla histolojik olarak konulsa da, ekokardiyografi ve kardiyak manyetik rezonans (CMR) gibi görüntüleme yöntemleri özellikle kalp tutulumu açısından değerlidir. Tedavide amaç, altta yatan inflamatuar hastalığın kontrol altına alınması ve SAA düzeylerinin düşürülmesidir. Kolşisin, NSAİİ’ler ve kortikosteroidler gibi geleneksel tedavilere ek olarak; anti-TNF, anti-IL-1 ve anti-IL-6 ajanlar gibi hedefe yönelik biyolojik tedaviler de umut vadetmektedir. Böbrek tutulumu olan olgularda destek tedavileri, diyaliz veya böbrek nakli gerekebilir. Ancak kalp tutulumu olan hastalarda, özellikle nakil sonrası mortalite oranları yüksektir. Güncel görüntüleme teknikleri ve biyolojik tedavilere rağmen, amiloid birikimlerinin tamamen ortadan kaldırılması halen mümkün değildir. Erken tanı ve uygun tedavi, hastalık seyrini olumlu yönde etkileyebilir.
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