[A rare manifestation of a cystic lung disease with high suspicion of pulmonary light chain deposition disease (LCDD)].

IF 1.7
Pneumologie (Stuttgart, Germany) Pub Date : 2026-03-01 Epub Date: 2025-09-23 DOI:10.1055/a-2694-6178
Nina Sicker, Sabine Gaerditz, Florian Geismann, Okka Hamer, Christoph Lerzer, Arno Mohr, Stefanie Scharf, Maximilian Malfertheiner
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Abstract

This case report describes a rare form of cystic lung disease, strongly suspected to be a pulmonary manifestation of light chain deposition disease (LCDD). Pulmonary light chain deposition disease (PLCDD) is an uncommon form of cystic lung disease, presenting with extensive bilateral cysts and nodules. Definitive diagnosis requires histopathological confirmation, although this may not always be feasible in cases of respiratory compromise. The diagnosis of LCDD should be considered when the clinical findings are atypical for other cystic lung diseases, the HRCT morphology suggests nodular-cystic amyloidosis (HRCT with cysts and nodules, nodules also involving the walls of the cysts, predominantly located in the periphery of the lower lung areas) or when monoclonal free light chains, particularly kappa chains, are detected in serum and/or urine.

【囊性肺疾病的罕见表现,高度怀疑肺轻链沉积病(LCDD)】。
本病例报告描述了一种罕见的囊性肺疾病,强烈怀疑是轻链沉积病(LCDD)的肺部表现。肺轻链沉积病(PLCDD)是一种罕见的囊性肺疾病,表现为广泛的双侧囊肿和结节。明确的诊断需要组织病理学证实,尽管这在呼吸系统损伤的病例中可能并不总是可行的。当临床表现与其他囊性肺疾病不典型时,应考虑LCDD的诊断,HRCT形态提示结节性囊性淀粉样变性(HRCT表现为囊肿和结节,结节也累及囊肿壁,主要位于下肺区外围),或当血清和/或尿液中检测到单克隆游离轻链,特别是kappa链时。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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