Sjögren Syndrome with Interstitial Lung Disease: What to Do When Immunosuppressive Agents Have Not Worked

This protocol applies to patients with Sjögren syndrome who have interstitial lung disease — specifically non-specific interstitial pneumonitis (NSIP), organising pneumonitis, or lymphoid interstitial pneumonitis — representing active systemic involvement, and who have not reached the treatment target on conventional immunosuppressive therapy.

Clinical Scenario

The patient has Sjögren syndrome with interstitial lung disease (NSIP, organising pneumonitis, or lymphoid interstitial pneumonitis) as an expression of active systemic disease. Treatment of systemic disease is tailored to organ-specific severity as defined by the ESSDAI.

Previous Line: Escalation Trigger

The prior treatment line used immunosuppressive agents — leflunomide, methotrexate, azathioprine, mycophenolate, or cyclophosphamide — targeting a reduction of ≥3 points in the global ESSDAI score. This protocol is indicated when that ESSDAI target has not been achieved.

Next-Step Treatment Approach

For severe, refractory systemic Sjögren syndrome with ILD, the approach involves rituximab, an intravenous biologic agent administered as a short defined course. The precise schedule and full clinical protocol are available in the complete regimen.

Target: ESSDAI reduction ≥3 points

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/annrheumdis-2019-216114

Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.
ILD with NHYA III/IV.
The use of rituximab may be considered in patients with severe, refractory systemic disease.
Rituximab: rituximab 1 g/15 days (x2).
With respect to the definition of the therapeutic response in systemic SjS, the TF recommends using a reduction of ≥3 points in the global ESSDAI score.

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