Sjögren Syndrome with Severe Autoimmune Thrombocytopenia When Glucocorticoids Have Not Worked

This protocol applies to patients with Sjögren syndrome who have developed severe autoimmune thrombocytopenia — a platelet count below 50,000/mm³ — and whose condition has not responded adequately to a glucocorticoid-based initial regimen.

Clinical Situation

The targeted population carries Sjögren syndrome alongside severe autoimmune thrombocytopenia (platelet count < 50,000/mm³). This places the patient in a high-risk haematological category that requires a structured escalation strategy.

After prior treatment failed

Reason for Escalation

The preceding step — glucocorticoids (oral or intravenous methylprednisolone) — was the first-line intervention. Escalation to this protocol is indicated when that approach fails to achieve a reduction of ≥3 points in the global ESSDAI score, indicating insufficient control of systemic disease activity.

Treatment Approach (Overview Only)

The escalation strategy in this setting involves a targeted monoclonal antibody-based therapy — the complete regimen, alternative options, and sequencing are defined in the full structured protocol.

Treatment Goal

The primary therapeutic target is a reduction of ≥3 points in the global ESSDAI (EULAR Sjögren's Syndrome Disease Activity Index) score, reflecting meaningful improvement in systemic disease activity.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/annrheumdis-2019-216114

Severe haemolytic anaemia (<80 g/dL, <50 x10^9/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.

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