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  • Sjögren’s syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient

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Case Reports: Unusual association of diseases/symptoms
Sjögren’s syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient
  1. Matthew Steward1,2,
  2. Johannes H Yu2 and
  3. Michael A Gibbons1
  1. 1Department of Respiratory Medicine, Royal Devon and Exeter NHS Foundation Trust, Exeter, Devon, UK
  2. 2College of Medicine and Health, University of Exeter Medical School, Exeter, Devon, UK
  1. Correspondence to Dr Matthew Steward; matt.steward1{at}nhs.net

Abstract

A man in his 70s presents with 12 months of progressive dyspnoea, sicca symptoms and Raynaud’s phenomenon. Serological testing and tear duct biopsy confirm Sjögren’s syndrome (SS). Bilateral nodular-cystic appearances highly suggestive of lymphoid interstitial pneumonia (LIP) are noted on high-resolution computed tomography (HRCT), supported by a 40% lymphocytosis on bronchoalveolar lavage.

Biopsy of a non-characteristic additional pulmonary nodule diagnoses light chain deposition disease (LCDD). Extrapulmonary organ involvement is excluded. Pulmonary function tests are well-preserved, and the patient is kept under active surveillance without requiring immunomodulatory treatment.

LIP and LCDD both have a strong association with SS. Identification of these disease associations is crucial as they may result in multiorgan involvement or progression to haematological malignancy. This is the first case published in the literature and highlights that a pragmatic approach to investigations can avoid unnecessary procedures, and that treatment may be guided by symptomology.

  • Interstitial lung disease
  • Connective tissue disease
  • Sjogren's syndrome

https://doi.org/10.1136/bcr-2022-249747

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    Footnotes

    • MS and JHY are joint first authors.

    • Contributors JHY and MS wrote, edited and finalised the manuscript. MS contributed to the revisions of the text. MAG conceived the project and edited the final manuscript. All authors agreed on the final edit of the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.