Systemic lupus erythematosus
ICD-10 M32 · ICD-11 4A40.0

SLE with Severe Autoimmune Thrombocytopenia — When First-Line Acute Therapy Has Not Sustained Platelet Count Above 30,000/mm³

This protocol applies to patients with systemic lupus erythematosus who have severe autoimmune thrombocytopenia — a platelet count below 20,000–30,000/mm³ — and in whom first-line acute treatment has not achieved a sustained platelet count above 30,000/mm³.

First-Line Therapy — Failure Condition

Acute treatment with high-dose glucocorticoids (including intravenous methylprednisolone pulses), with or without intravenous immunoglobulin G, rituximab, or high-dose intravenous cyclophosphamide did not achieve the target of sustained platelet counts above 30,000/mm³. This protocol defines the structured next step.

Treatment Goal

Sustained platelet count above 30,000/mm³.

Approach

The full protocol specifies a maintenance immunomodulating therapy strategy — including agent selection and sequencing — tailored to this clinical situation. See the structured regimen for details.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/ard-2023-224762

For acute treatment of severe autoimmune thrombocytopenia, high-dose glucocorticoids (including pulses of intravenous methylprednisolone) (4/C), with or without intravenous immunoglobulin G (4/C), and/or rituximab (2b/B), and/or high-dose intravenous cyclophosphamide (4/C), followed by maintenance therapy with rituximab (2b/B), azathioprine (2b/C), mycophenolate (2b/C), or cyclosporine (4/C) should be considered.

A platelet number of 20–30 000/mm³ is typically used as the cut-off, below which therapy is indicated.

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