Pulmonary light-chain deposition disease: CT and pathology findings in nine patients

•Nine cases of pulmonary light chain deposition disease with high-resolution CT.•CT scans assessed for abnormal features.•Clinical data and histopathological findings obtained.•All patients had thin-walled cysts on CT, often with traversing vessels.•CT features of disease in this group are cysts, nodules and an indolent course.

AimTo review the clinical features of nine patients with pulmonary light-chain deposition disease (LCDD) and record their high-resolution CT (HRCT) and histopathological findings.Materials and methodsPatients with a diagnosis of LCDD on lung biopsy specimen were retrospectively identified. The HRCT characteristics of nodules, cysts, and ancillary findings; change at follow-up; and histopathological findings were documented.ResultsFeatures common to all nine cases were thin-walled cysts. In seven cases, vessels traversing the cysts were identified. The majority of patients (8/9) had at least one pulmonary nodule. There was no zonal predominance of either cysts or nodules. The disease appeared stable in the majority of cases with no serial change in HRCT appearances (5/6 cases with follow-up data, mean duration 29 months).ConclusionTo the authors' knowledge, this is the largest series of pulmonary LCDD patients in the literature, and the first systematic assessment of HRCT findings. Pulmonary cysts are a unifying feature, usually with pulmonary nodules, and serial change on HRCT is unusual.