Sjögren's disease & overlap diseases

Lymphoid interstitial pneumonia in Sjögren disease: clinical course and comparison with other Interstitial Lung Disease (ILD) patterns

Rheumatology (Oxford)Published Retrospective cohort study

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)
GroupValueDLCO median (IQR)
LIPmedian 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

OutcomeLIPnon-LIPP value
ILD preceding SjD diagnosis1/1130/44P < 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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