Table 2 Treatment of CAPS used in the described patient

First line treatment

Anticoagulation

High molecular weight heparin (HMWH)

LMWH

Oral anticoagulant (warfarin, INR > 3)

Glucocorticoids

Methylprednisolone 1 g i.v. for three consecutive days

Oral prednisone, 1 mg/kg/day (60 mg/day)

Oral prednisolone tapered to 20 mg within 6 weeks

TPE

5 sessions with the frequency every other day

1,5 of total plasma volume per session

Replacement fluid: 4% albumin solution and FFP

Immunoglobulin

5 doses of IgG 0,4 g/kg/day

Second line treatment

Rituximab

2 g in three separate doses (600–800-600 mg) in two-month intervals