Sjögren Syndrome with Severe Autoimmune Haemolytic Anaemia When Glucocorticoids Have Not Achieved Disease Control

Clinical scenario

This protocol addresses patients with Sjögren syndrome who have developed severe autoimmune haemolytic anaemia — defined by a haemoglobin below 80 g/dL — and whose haematological disease has not responded adequately to first-line glucocorticoid therapy.

Previous line — goals not met

First-line treatment with glucocorticoids (oral for moderate disease, or intravenous methylprednisolone pulses followed by oral for severe disease) did not achieve a reduction of ≥3 points in the global ESSDAI score. Failure to reach this threshold on the haematological–autoimmune haemolytic anaemia line is the trigger for escalation to this protocol.

Next-line approach

This protocol involves targeted biological or immunological infusion therapy. The treatment goal remains a reduction of ≥3 points in the global ESSDAI score. The specific agents and full administration details are available in the complete regimen.

Instant Access to Structured Evidence-Based Regimens

References

Severe haemolytic anaemia (<80 g/dL, <50 x10⁹/L); severe autoimmune thrombocytopenia (<50 000/mm³).
Rituximab: rituximab 1 g/15 days (x2). IVIg: intravenous immunoglobulins 0.4–2 g/kg 5 days.
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.

DOI: 10.1136/annrheumdis-2019-216114

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