Sjögren's disease & overlap diseases
Lymphoid interstitial pneumonia in Sjögren disease: clinical course and comparison with other Interstitial Lung Disease (ILD) patterns
Authors' conclusion
LIP defines a distinct, immunologically active phenotype within the spectrum of SjD-ILD, characterized by greater extrapulmonary systemic involvement and serologic markers of B cell hyperactivity, but limited pulmonary functional impact.
Study context
- Population
- Sjögren Disease (SjD) patients with High-resolution Computed Tomography (HRCT)-confirmed ILD followed in Pisa Rheumatology Unit (January 2019-November 2024)M: F = 9:46
- Participants
- 55 patients
- Exposure
- SjD-associated lymphoid interstitial pneumonia (LIP) vs other SjD-ILD patterns
- LIPn = 11
- non-LIPn = 44reference
- Outcome
- Clinical course and outcome of SjD-associated LIP, compared with other SjD-ILD patterns
Methodological detailssingle-centre, multidisciplinary ILD pattern classification
- Single-centre
- Multidisciplinary ILD pattern classification
Main finding
As reported in the abstract
Clinical course of SjD-LIP · median 5-years follow-up (IQR 2-7)
| Group | Value | DLCO median (IQR) |
|---|---|---|
| LIP | median 101% (IQR 98–105) | 76% (75-81) |
LIP
Valuemedian 101% (IQR 98–105)
DLCO median (IQR)76% (75-81)
- LIP sex ratio F: M
- 11:0
Observational data: associations, not effects.
Further findings
| Outcome | LIP | non-LIP | P value |
|---|---|---|---|
| ILD preceding SjD diagnosis | 1/11 | 30/44 | P < 0.001 |
- Clinical-serologic features, LIP vs non-LIP
- P < 0.001
- Clinical-serologic features, LIP vs non-LIP
- P = 0.012
- Clinical-serologic features, LIP vs non-LIP
- P = 0.001
- Clinical-serologic features, LIP vs non-LIP
- P = 0.023
- Clinical-serologic features, LIP vs non-LIP
- P < 0.001
- Clinical-serologic features, LIP vs non-LIP
- P = 0.035
- Clinical-serologic features, LIP vs non-LIP
- P = 0.009
- Pulmonary function, non-LIP vs LIP
- P = 0.049
- Pulmonary function, non-LIP vs LIP
- P = 0.036
Original publication
Lymphoid interstitial pneumonia in Sjögren disease: clinical course and comparison with other ILD patterns. Rheumatology (Oxford). Published 22 June 2026. doi:10. 1093/ rheumatology/ keag321
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