{
  "abstract": "Background\n\nInterstitial 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\n\nA 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\n\nCystic 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.",
  "abstract_shown": true,
  "affiliations": [
    "Rheumatology Department, Traditional Chinese Medicine Hospital of Meishan, Meishan, Sichuan, China.",
    "Department of Respiratory and Critical Care Medicine, Traditional Chinese Medicine Hospital of Meishan, Meishan, Sichuan, China.",
    "Department of Respiratory and Critical Care Medicine, West China Hospital, Chengdu, Sichuan, China.",
    "Pathology Department, Traditional Chinese Medicine Hospital of Meishan, Meishan, Sichuan, China.",
    "Department of Respiratory and Critical Care Medicine, Traditional Chinese Medicine Hospital of Meishan, Meishan, Sichuan, China. 404660951@qq.com."
  ],
  "article_type": null,
  "arxiv": null,
  "author_total": 10,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Jinzhou Chen"
    },
    {
      "affs": [
        1
      ],
      "name": "Songtao Ren"
    },
    {
      "affs": [
        1
      ],
      "name": "Jing Huang"
    },
    {
      "affs": [
        2
      ],
      "name": "Ting Yang"
    },
    {
      "affs": [
        1
      ],
      "name": "Guangbing Lu"
    },
    {
      "affs": [
        1
      ],
      "name": "Hua Fang"
    },
    {
      "affs": [
        3
      ],
      "name": "Xiaojing Deng"
    },
    {
      "affs": [
        1
      ],
      "name": "Bo Zhang"
    },
    {
      "affs": [
        0
      ],
      "name": "Sixian Liu"
    },
    {
      "affs": [
        4
      ],
      "name": "Liangdong Tian"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/95k2zydman8aa.bib",
  "cited_by": 0,
  "doi": "10.1186/s12890-026-04528-0",
  "fulltext_url": "https://europepmc.org/articles/PMC13679215",
  "id": "95k2zydman8aa",
  "issns": [
    "1471-2466"
  ],
  "landing_url": "https://europepmc.org/article/MED/42894175",
  "language": "eng",
  "licence": "CC-BY-NC-ND",
  "licence_label": "CC BY-NC-ND",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by-nc-nd/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "lcc",
      "RC705-779"
    ],
    [
      "openalex",
      "2740|Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis"
    ]
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  "openalex": "W7170033959",
  "pdf_url": "https://europepmc.org/articles/PMC13679215?pdf=render",
  "pmcid": "PMC13679215",
  "pmid": "42894175",
  "published": "2026-07-01",
  "publisher": "BMC",
  "ris": "https://research.draconitic.com/paper/95k2zydman8aa.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:19:42Z",
      "native_id": "MED:42894175",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42894175"
    },
    {
      "first_seen": "2026-10-30T13:45:34Z",
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  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2740|Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis",
      "subfield": "general"
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  ],
  "title": "Cystic pulmonary amyloidosis with lymphoid interstitial pneumonia in Sjögren's disease: a case report.",
  "title_text": "Cystic pulmonary amyloidosis with lymphoid interstitial pneumonia in Sjögren's disease: a case report.",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T13:45:34Z",
  "url": "https://research.draconitic.com/paper/95k2zydman8aa",
  "venue": "BMC pulmonary medicine",
  "versions": []
}