Thirumurthy Hem Sunder, Sekar Aravind, Suri Vikas, Lal Anupam, Nada Ritambra
Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, IND.
Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, IND.
Cureus. 2025 Jun 27;17(6):e86863. doi: 10.7759/cureus.86863. eCollection 2025 Jun.
Amyloidosis refers to a heterogeneous group of disorders characterized by the extracellular deposition of insoluble fibrillar proteins, leading to tissue damage and functional impairment. Among these, serum amyloid A (SAA) amyloidosis, previously termed secondary amyloidosis, arises in the context of chronic inflammatory conditions. While common causes include autoimmune diseases and chronic infections, the underlying etiology remains unidentified in a subset of cases. We report a rare case of SAA-associated amyloidosis in a 29-year-old male with a history of childhood-onset cystic bronchiectasis. The patient presented with progressive renal dysfunction and was diagnosed with systemic amyloidosis based on histopathological evaluation. Common autoimmune and infectious etiologies were excluded during life. An autopsy revealed alpha-1 antitrypsin deficiency as the underlying cause of chronic pulmonary inflammation, manifesting as cystic bronchiectasis, which likely triggered sustained elevation of serum amyloid A and subsequent amyloid deposition.
淀粉样变性是指一组异质性疾病,其特征是不溶性纤维状蛋白在细胞外沉积,导致组织损伤和功能障碍。其中,血清淀粉样蛋白A(SAA)淀粉样变性,以前称为继发性淀粉样变性,发生于慢性炎症状态。常见病因包括自身免疫性疾病和慢性感染,但在一部分病例中,潜在病因仍不明。我们报告一例罕见的SAA相关性淀粉样变性病例,患者为一名29岁男性,有儿童期起病的囊性支气管扩张病史。该患者出现进行性肾功能不全,基于组织病理学评估被诊断为系统性淀粉样变性。生前排除了常见的自身免疫和感染性病因。尸检显示α-1抗胰蛋白酶缺乏是慢性肺部炎症的潜在病因,表现为囊性支气管扩张,这可能引发血清淀粉样蛋白A持续升高及随后的淀粉样沉积。