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

Treatment of Sjögren Syndrome with Severe Autoimmune Haemolytic Anaemia (Haemoglobin <80 g/dL)

Clinical Scenario

This protocol covers patients with Sjögren syndrome who present with severe autoimmune haemolytic anaemia, defined by a haemoglobin level below 80 g/dL. This level of haematological compromise represents a high-severity systemic manifestation requiring prompt, structured intervention.

Specific Situation

Haemoglobin falling below 80 g/dL signals severe haemolytic involvement in the context of active Sjögren syndrome. Addressing both the acute haematological deficit and the underlying autoimmune drive is central to management at this threshold.

Treatment Approach (Partial Overview)

Management involves glucocorticoid-based therapy, with the specific approach varying according to severity — the complete regimen is available via the full protocol.

Treatment Goal

The primary therapeutic target is a reduction of at least 3 points in the global ESSDAI score, reflecting meaningful control of systemic disease activity.

Instant Access to Structured Evidence-Based Regimens

References

  1. Severe haemolytic anaemia (<80 g/dL, <50 ×109/L); severe autoimmune thrombocytopenia (<50 000/mm3).
  2. GCs should be used at the minimum dose and length of time necessary to control active systemic disease, administering pulses of methylprednisolone followed by doses of 0.5 mg/kg/d or lower as induction therapy in severe presentations, and doses <0.5 mg/kg/d in moderate/less-severe presentations, with a final target of withdrawing GCs in inactive patients as soon as possible or at least trying to target a maintenance dose of 5 mg/daily or less with the aid of GC-sparing immunosuppressive agents.
  3. 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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