Sjögren syndrome
ICD-10 M35.0 · ICD-11 4A43.2

Sjögren Syndrome with Interstitial Lung Disease: What to Do When Glucocorticoids Are Not Enough

This protocol is for patients with Sjögren syndrome complicated by interstitial lung disease — NSIP, organising pneumonitis, or lymphoid interstitial pneumonitis — alongside active systemic disease, in whom first-line glucocorticoid therapy has not achieved adequate disease control.

Clinical scenario

Sjögren syndrome with interstitial lung disease (NSIP, organising pneumonitis, or lymphoid interstitial pneumonitis) in the setting of active systemic disease. Organ-specific severity is assessed using ESSDAI domain definitions to guide the treatment approach.

Why this next-line protocol applies

Previous therapy: Glucocorticoids were used as the first-line treatment for this pulmonary manifestation.

Failure condition: Glucocorticoid therapy did not achieve a reduction of ≥3 points in the global ESSDAI score — the defined threshold for adequate therapeutic response in systemic Sjögren syndrome.

When that target is not met, this protocol defines the next clinical step.

Next-line approach (partial overview)

The next step involves a transition to immunosuppressive therapy. Several agents within this class are considered options at this stage, with no single agent currently preferred over others based on comparative evidence. The specific agent choice, sequencing, and monitoring are detailed in the full protocol.

Treatment goal

The primary target is a reduction of ≥3 points in the global ESSDAI score, indicating clinically meaningful improvement in systemic disease activity.

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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 lack of head-to-head studies comparing the efficacy and safety profile of immunosuppressive agents in primary SjS-2002 (leflunomide, methotrexate, azathioprine, mycophenolate, cyclophosphamide) does not permit a recommendation on the use of one agent over another.

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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