Cystic pulmonary amyloidosis with lymphoid interstitial pneumonia in Sjögren's disease: a case report.
5 affiliations
- 1Rheumatology Department, Traditional Chinese Medicine Hospital of Meishan, Meishan, Sichuan, China.
- 2Department of Respiratory and Critical Care Medicine, Traditional Chinese Medicine Hospital of Meishan, Meishan, Sichuan, China.
- 3Department of Respiratory and Critical Care Medicine, West China Hospital, Chengdu, Sichuan, China.
- 4Pathology Department, Traditional Chinese Medicine Hospital of Meishan, Meishan, Sichuan, China.
- 5Department of Respiratory and Critical Care Medicine, Traditional Chinese Medicine Hospital of Meishan, Meishan, Sichuan, China. [email protected].
Abstract
Background
Interstitial lung diseases are commonly seen in association with Sjögren's disease, especially lymphoproliferative disorders, such as lymphoid interstitial pneumonia. In occasional and rare cases, pulmonary amyloidosis with cysts, nodules, and/or ground-glass opacites as radiological features, can also become a complication of Sjögren's disease. On the other hand, diffuse cystic lung disease, without nodules, concomitant with chylous fluid accumulation in female leads to initial suspicion of lymphangioleiomyomatosis. Interestingly, it's challenging to differentiate if diffuse cystic lung lesion absent of nodules, chylous fluid accumulation and Sjögren's disease happen simultaneously.Case presentation
A 34-year-old female, with diffuse cystic lung disease, chylous reflux lymphedema and Sjögren's disease, was eventually diagnosed with cystic pulmonary amyloidosis with lymphoid interstitial pneumonia, secondary to Sjögren's disease, and chylous reflux lymphedema as comorbidity. The case presented with atypical pulmonary amyloidosis radiological features of lung lesion, where diffuse cysts existed without nodules or ground-glass opacites, apart from the scarcely seen size of the biggest cyst. The patient was diagnosed with CPA with LIP, secondary to SjD. Clinical symptoms were relieved after thoracoscopic lobectomy of the right lower lobe, where located the biggest cyst. The immunosuppressants that she had been taking were recommended again for disease stabilization.Conclusion
Cystic pulmonary amyloidosis is a rare complication of Sjögren's disease, while lymphoid interstitial pneumonia is comparatively well-known. There can be cystic pulmonary amyloidosis cases where the lung lesion only consists of diffuse, thin-walled, and variably-sized cysts, with the absence of nodules or ground-glass opacities, which warrants a lung biopsy. Clinicians should be alerted to the cyst-only radiological features of the cystic pulmonary amyloidosis with Sjögren's disease.
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BibTeX
@article{chen2026cystic,
title = {{Cystic pulmonary amyloidosis with lymphoid interstitial pneumonia in Sjögren's disease: a case report.}},
author = {Jinzhou Chen and Songtao Ren and Jing Huang and Ting Yang and Guangbing Lu and Hua Fang and Xiaojing Deng and Bo Zhang and Sixian Liu and Liangdong Tian},
journal = {BMC pulmonary medicine},
year = {2026},
month = jul,
doi = {10.1186/s12890-026-04528-0},
url = {https://europepmc.org/article/MED/42894175},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Cystic pulmonary amyloidosis with lymphoid interstitial pneumonia in Sjögren's disease: a case report.
AU - Jinzhou Chen
AU - Songtao Ren
AU - Jing Huang
AU - Ting Yang
AU - Guangbing Lu
AU - Hua Fang
AU - Xiaojing Deng
AU - Bo Zhang
AU - Sixian Liu
AU - Liangdong Tian
PY - 2026
DA - 2026/07/01
JO - BMC pulmonary medicine
DO - 10.1186/s12890-026-04528-0
UR - https://europepmc.org/article/MED/42894175
AB - Background Interstitial lung diseases are commonly seen in association with Sjögren's disease, especially lymphoproliferative disorders, such as lymphoid interstitial pneumonia. In occasional and rare cases, pulmonary amyloidosis with cysts, nodules, and/or ground-glass opacites as radiological features, can also become a complication of Sjögren's disease. On the other hand, diffuse cystic lung disease, without nodules, concomitant with chylous fluid accumulation in female leads to initial suspicion of lymphangioleiomyomatosis. Interestingly, it's challenging to differentiate if diffuse cystic lung lesion absent of nodules, chylous fluid accumulation and Sjögren's disease happen simultaneously.Case presentation A 34-year-old female, with diffuse cystic lung disease, chylous reflux lymphedema and Sjögren's disease, was eventually diagnosed with cystic pulmonary amyloidosis with lymphoid interstitial pneumonia, secondary to Sjögren's disease, and chylous reflux lymphedema as comorbidity. The case presented with atypical pulmonary amyloidosis radiological features of lung lesion, where diffuse cysts existed without nodules or ground-glass opacites, apart from the scarcely seen size of the biggest cyst. The patient was diagnosed with CPA with LIP, secondary to SjD. Clinical symptoms were relieved after thoracoscopic lobectomy of the right lower lobe, where located the biggest cyst. The immunosuppressants that she had been taking were recommended again for disease stabilization.Conclusion Cystic pulmonary amyloidosis is a rare complication of Sjögren's disease, while lymphoid interstitial pneumonia is comparatively well-known. There can be cystic pulmonary amyloidosis cases where the lung lesion only consists of diffuse, thin-walled, and variably-sized cysts, with the absence of nodules or ground-glass opacities, which warrants a lung biopsy. Clinicians should be alerted to the cyst-only radiological features of the cystic pulmonary amyloidosis with Sjögren's disease.
SN - 1471-2466
ER -