Department of Internal medicine, Division of Rheumatology, Ege University Faculty of Medicine, Izmir, Turkey.
Department of Pathology, Ege University Faculty of Medicine, Izmir, Turkey.
Int J Rheum Dis. 2024 Jan;27(1):e14984. doi: 10.1111/1756-185X.14984. Epub 2023 Nov 28.
Systemic AA amyloidosis is associated with poorly controlled chronic inflammatory disorders. Chronic infections and inflammatory arthritis are the most common causes; however, they can also rarely occur as a complication of neoplastic disorders. The development of AA amyloidosis secondary to paraganglioma, which is a rare type of tumor, has rarely been reported in the literature. In this case, an 85-year-old female patient with a glomus tumor in the neck, who has been followed up over 50 years, applied with complaints of loss of appetite, nausea, and diarrhea for 5-6 months. While evaluating the patient, who had high levels of acute phase reactants, amyloidosis was diagnosed by salivary gland biopsy. No other cause was found to explain amyloidosis. The patient, who could not tolerate treatment with colchicine and azathioprine, is successfully treated with the interleukin-1 inhibitor anakinra. A rare relationship, systemic AA amyloidosis, which is thought to have developed as a result of long-standing jugular paraganglioma, is presented in this article. In addition, publications showing an association between paragangliomas and amyloidosis were reviewed.
系统性 AA 淀粉样变性与慢性炎症性疾病控制不良有关。慢性感染和炎性关节炎是最常见的原因,但也可能很少作为肿瘤性疾病的并发症发生。副神经节瘤继发的 AA 淀粉样变性是一种罕见的肿瘤类型,在文献中很少有报道。在这种情况下,一名 85 岁的女性患者,颈部有血管球瘤,已随访超过 50 年,出现食欲不振、恶心和腹泻 5-6 个月的症状就诊。在评估患者时,发现其急性期反应物水平较高,通过唾液腺活检诊断为淀粉样变性。未发现其他原因解释淀粉样变性。由于无法耐受秋水仙碱和巯嘌呤治疗,该患者成功接受白细胞介素-1 抑制剂阿那白滞素治疗。本文介绍了一种罕见的关系,即认为是由于长期存在的颈静脉副神经节瘤引起的系统性 AA 淀粉样变性。此外,还回顾了显示副神经节瘤与淀粉样变性之间关联的出版物。