Peshin Supriya, Kulkarni Vaishnavi, Bashir Faizan, Iftekhar Rafi, Kapadia Jaimin, Donovan Mabe, Nader Elie
Department of Internal Medicine Norton Community Hospital Norton Virginia USA.
School of Medicine Shiraz University of Medical Sciences Shiraz Iran.
Respirol Case Rep. 2025 May 13;13(5):e70211. doi: 10.1002/rcr2.70211. eCollection 2025 May.
Pulmonary amyloidosis is an uncommon but clinically significant disorder characterised by extracellular deposition of misfolded amyloid fibrils in lung tissue, either as part of systemic disease or as a localised entity. We present a case of a 62-year-old man with coal workers' pneumoconiosis and a 40 pack-year smoking history, who developed progressive dyspnea, chronic cough, and weight loss. Initial chest CT revealed bilateral thin-walled pulmonary cysts without nodularity. Over several years, the cystic lesions evolved, with interval development of a mural nodule in the right lower lobe. PET imaging was non-avid, and bronchoscopy yielded nondiagnostic cytology. A left lower lobe wedge resection, performed for a surgically accessible stable cyst, confirmed localised AL (kappa) nodular pulmonary amyloidosis on Congo red staining. Hematologic workup demonstrated an elevated kappa/lambda free light chain ratio without evidence of systemic or cardiac involvement. The patient remains clinically stable under multidisciplinary surveillance. This case illustrates the diagnostic complexity of pulmonary amyloidosis and underscores the importance of including it in the differential diagnosis of cystic lung disease, particularly in the setting of occupational exposure and indeterminate imaging.
肺淀粉样变性是一种罕见但具有临床意义的疾病,其特征是错误折叠的淀粉样纤维在肺组织中细胞外沉积,可作为全身性疾病的一部分或作为局部病变。我们报告一例62岁男性,有煤工尘肺病史及40年40包年的吸烟史,出现进行性呼吸困难、慢性咳嗽和体重减轻。初始胸部CT显示双侧薄壁肺囊肿,无结节。数年间,囊性病变进展,右肺下叶出现壁结节。PET成像无摄取,支气管镜检查细胞学检查未明确诊断。因手术可及的稳定囊肿行左下叶楔形切除术,刚果红染色证实为局限性AL(κ)结节性肺淀粉样变性。血液学检查显示κ/λ游离轻链比值升高,无全身或心脏受累证据。患者在多学科监测下临床情况稳定。该病例说明了肺淀粉样变性的诊断复杂性,并强调了在囊性肺疾病鉴别诊断中考虑该病的重要性,尤其是在职业暴露和影像学表现不明确的情况下。