The other connective tissue disease-associated interstitial lung diseases: Sjogren's syndrome, mixed connective tissue disease, and systemic lupus erythematosus

Curr Opin Pulm Med. 2021 Sep 1;27(5):388-395. doi: 10.1097/MCP.0000000000000791.


Purpose of review: We review the clinical manifestations of three less common connective tissue disease (CTD)-associated interstitial lung diseases (ILDs): Sjogren's syndrome (SjS), mixed CTD (MCTD), and systemic lupus erythematosus (SLE).

Recent findings: SjS is classically associated with lymphocytic interstitial pneumonia and cystic lung disease, but the most common type of ILD in Sjogren's patients is nonspecific interstitial pneumonia. ILD is prevalent in MCTD and associated with worse survival. SLE-associated ILD, while rare, is more common in those with CTD overlap syndromes. Regardless of underlying cause, a subset of patients with fibrotic CTD-associated ILD develop a progressive course for which antifibrotic agents and lung transplantation should be considered.

Summary: An understanding of the characteristics of ILD in SjS, MCTD, and SLE is important for the pulmonary specialist. Future research should identify risk factors for progression and develop additional treatment modalities for both CTD-related autoimmune features and progressive ILD.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Connective Tissue Diseases* / complications
  • Humans
  • Lung Diseases, Interstitial* / etiology
  • Lupus Erythematosus, Systemic* / complications
  • Mixed Connective Tissue Disease* / complications
  • Sjogren's Syndrome* / complications