Treatment of Antiphospholipid Syndrome in Catastrophic Antiphospholipid Syndrome

Catastrophic antiphospholipid syndrome (CAPS) is a severe clinical presentation of antiphospholipid syndrome (APS) that requires a structured, evidence-based treatment approach tailored to this specific scenario.

Clinical scenario

This protocol applies to patients with catastrophic antiphospholipid syndrome — a distinct, high-acuity form of APS in which a specific combination treatment strategy is indicated.

Treatment approach (partial)

Management involves a combination approach incorporating anticoagulation, immunosuppression, and blood purification strategies, with the potential addition of targeted biological agents directed at specific immune pathways — the full sequence and criteria are detailed in the protocol.

Instant Access to Structured Evidence-Based Regimens

References

For first-line treatment of patients with CAPS, combination therapy with glucocorticoids, heparin and plasma exchange or intravenous immunoglobulins is recommended over single agents or other combinations of therapies.
In patients with refractory CAPS, B cell depletion (eg, rituximab) or complement inhibition (eg, eculizumab) therapies may be considered.

DOI: 10.1136/annrheumdis-2019-215213

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