从呼吸科视角看肺轻链沉积病

Pulmonary light chain deposition disease from a respiratory physician′s perspective

肺轻链沉积病(pulmonary light chain deposition disease,PLCDD)是一种罕见的单克隆免疫球蛋白沉积病,由特异性的免疫球蛋白轻链在肺组织沉积引起,早期临床表现轻微,肺部影像学表现为多发结节及薄壁囊腔,病理显示组织中嗜酸性无定形蛋白样物质沉积,刚果红染色阴性。肺轻链沉积病的确切病理机制尚不明确,临床表现缺乏特异性,存在的问题包括诊断困难、治疗方案的选择和效果评价、病情监测和预后判断标准不明确等方面。需要更多的研究来阐明PLCDD的病理机制、标准化的诊疗流程、治疗效果评估及预后的相关因素。

Pulmonary light chain deposition disease(PLCDD)is a rare monoclonal immunoglobulin deposition disease characterized by the deposition of specific immunoglobulin light chains in lung tissue.In its early stages,PLCDD presents with mild clinical symptoms,while pulmonary imaging shows multiple nodules and thin-walled cysts.Pathologically,there is a deposition of eosinophilic amorphous protein-like material in the tissues,which stains negative for Congo red.The exact pathological mechanism of PLCDD remains unclear,and its clinical presentation lacks specificity.Challenges associated with this condition include difficulties in diagnosis,selection and evaluation of treatment options,lack of clear monitoring criteria,and standards for prognosis assessment.Further research is needed to elucidate the pathogenesis of PLCDD,to establish standardized diagnostic and therapeutic protocols,and to evaluate treatment efficacy and prognostic factors.