Elsevier

Clinical Radiology

Volume 70, Issue 5, May 2015, Pages 515-522
Clinical Radiology

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

https://doi.org/10.1016/j.crad.2015.01.002Get rights and content

Highlights

  • 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.

Aim

To 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 methods

Patients 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.

Results

Features 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).

Conclusion

To 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.

Introduction

Light-chain deposition disease (LCDD), characterized by the systemic accumulation of immunoglobulin light chains, is most commonly encountered in lymphoproliferative disorders but can also be associated with autoimmune conditions, whilst a few cases are idiopathic.1, 2 LCDD is recognized as a predominantly multisystem disease, with kidneys, liver, and heart the organs frequently affected. However, it is rarely encountered as a localized pulmonary disease.3

The literature on pulmonary LCDD is confined to small series, and reports are centred on its histopathological features and the distinction of LCDD from pulmonary amyloidosis, a disorder with strikingly similar morphology but different histochemical staining characteristics.3 However, imaging and outcome data for patients with LCDD are sparse, with details on no more than 12 cases in combined series to date.2, 4, 5, 6

The purpose of this study was to review a series of nine patients with LCDD to document their high-resolution CT (HRCT) findings and histopathological findings, as well as investigate serial change in patients with follow-up CT examinations.

Section snippets

Materials and methods

Patients with a diagnosis of pulmonary LCDD were retrieved from the archives of the histopathology department of the Royal Brompton and Harefield NHS Foundation Trust. Of the 10 cases identified, five cases were external referrals. Imaging for internal patients (n = 5) was available on the Trust PACS, and imaging for external patients (n = 5) was requested from the referring hospitals. CT images were obtained for all 10 patients, but one was excluded as it was of poor quality. Six of the nine

Clinical

Clinical data are given in Table 1. Ages ranged from 33–78 years, with a mean of 60.7 years, and a male:female ratio of 1:2. Five cases presented with incidental nodules whilst being investigated for other disorders. One of these patients had known renal LCDD. Reasons for initial thoracic imaging in the other four patients were: investigation for recurrent lower respiratory tract infections and shortness of breath; deteriorating lung function on a background of Sjögren's syndrome with known

Discussion

This series of patients with LCDD had a distinctive constellation of findings at CT that reflect the features seen at histology. In the present series, the majority of cases were associated with clonal lymphoid proliferation in the lungs, although one of the cases was associated with chronic lymphocytic leukaemia. Despite this clonality, the pulmonary morphology of LCDD in the present series appears to remain stable or be slowly progressive, although mean follow-up intervals are admittedly

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