A rare clinical case of systemic AA amyloidosis with cardiac involvement complicating ankylosing spondylitis: a case report

Leïla Barakat, Khadija Echchilali, Mina Moudatir, Hassan El Kabli, Yassine Ettagmouti, Mériem Haboub, Salim Arous, Mohamed Ghali Benouna, Abdenasser Drighil, Rachida Habbal, Meryame Azim, Asmae Mazti, Meriem Regragui, Nissrine Bennani Guebessi, Mehdi Karkouri
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Abstract

Ankylosing spondylitis (AS) is a type of chronic inflammation that is most prevalent in young adults and is characterized by an inflammatory enthesiopathy that gradually develops toward ossification and ankylosis. If inflammation is left unchecked, it can potentially lead to complications such as secondary amyloidosis, also known as AA amyloidosis, involving the deposition of amyloid serum A protein. Our case presents with a thyroid localization of AA amyloidosis which is secondary to this AS. Such a case has been described in only four cases in the literature. Cardiac localization of AA amyloidosis has been exceptionally described in the literature. We report the case of a young patient with severe AS complicated by secondary amyloidosis with thyroid, cardiac, and probably renal localization. He was treated with anti-TNF therapy, and his condition improved significantly. Our case presents several localizations of AA amyloidosis secondary to this AS. Although cardiac involvement is rare in secondary AA amyloidosis, it should always be screened for, even in a cardiacly asymptomatic patient.
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强直性脊柱炎并发心脏受累的系统性 AA 淀粉样变性的罕见临床病例:病例报告
强直性脊柱炎(AS)是一种慢性炎症,多发于青壮年,其特点是炎症性强直,逐渐发展为骨化和强直。如果炎症得不到控制,有可能导致并发症,如继发性淀粉样变性,也称为 AA 淀粉样变性,涉及淀粉样血清 A 蛋白的沉积。我们的病例是继发于这种强直性脊柱炎的甲状腺局部 AA 淀粉样变性。此类病例在文献中仅有四例。AA淀粉样变性的心脏局部病变在文献中鲜有描述。我们报告了一例年轻的重症强直性脊柱炎并发继发性淀粉样变性的病例,该病有甲状腺、心脏和可能的肾脏定位。他接受了抗肿瘤坏死因子治疗,病情明显好转。我们的病例显示了继发于这种强直性脊柱炎的 AA 淀粉样变性的几种定位。虽然心脏受累在继发性AA淀粉样变性中很少见,但即使是心脏无症状的患者也应进行筛查。
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