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

Sjögren Syndrome with Neutropenia and Active Systemic Disease: What to Do When G-CSF Has Not Worked

Clinical scenario

This protocol applies to patients with Sjögren syndrome who have neutropenia alongside active systemic disease. Treatment of systemic disease should be tailored to organ-specific severity, assessed through the ESSDAI framework.

Previous treatment — failure condition

When Granulocyte colony-stimulating factor (G-CSF) has been used for haematological involvement — specifically neutropenia — and has not achieved a reduction of ≥3 points in the global ESSDAI score, escalation to a different approach is indicated. This protocol defines the next step.

Next-step treatment approach (partial)

The next line involves consideration of immunosuppressive agents. Several options exist within this class; which to choose, and in what order, depends on the patient's individual profile. The complete selection criteria and sequencing are available in the full protocol.

Treatment goal

The primary therapeutic target is a reduction of ≥3 points in the global ESSDAI score — the accepted measure of meaningful systemic disease activity response in Sjögren syndrome.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/annrheumdis-2019-216114 View source ↗