How is cryopyrin associated periodic syndrome treated
Approach to the treatment of a patient who presents with cryopyrin associated periodic syndrome (CAPS)
Owing to the high mortality (30%–50%) in patients who develop CAPS, every effort should be made to prevent its development. APS patients and asymptomatic patients with aPL abs must have infections (including minor infections) treated aggressively and given prophylactic anticoagulation for surgery and in the postpartum period as outlined previously. Patients who develop CAPS can be treated as follows:
- • First-line therapy.
- – IV unfractionated heparin (not LMWH).
- – Methylprednisolone 1000 mg daily for 3 to 5 days followed by high-dose oral prednisone.
- • Second-line therapy.
- – Plasmapheresis daily for first 3 days. Replacement fluid should be albumin and not fresh frozen plasma unless patient also has TTP/HUS.
- – IVIG after plasmapheresis completed: 0.4 g/kg/day for 5 days.
- • Third-line therapy.
- – Cyclophosphamide and/or rituximab, especially if associated with active SLE.
- • Fourth-line therapy.
- – Eculizumab for complement inhibition. Complement activation is important in the pathogenesis of CAPS.
- – Other experimental: defibrotide.
Anticoagulation should be initiated as soon as possible when considered safe. Anticoagulation may need to be delayed if the patient is having life-threatening hemorrhagic complications (pulmonary hemorrhage, intracerebral hemorrhage) as part of their CAPS presentation. The combination of plasmapheresis, glucocorticoids, and anticoagulation (“triple therapy”) has decreased CAPS mortality by 33% compared with any one therapy alone.
