Cryofibrinogenemia — What to Do When First-Line Therapy Has Not Achieved Complete Clinical Improvement
This protocol addresses essential cryofibrinogenemia in the specific setting where the initial treatment approach has been tried but has not produced a complete response, and the question is what comes next.
Clinical Situation
Absence of cryoglobulinaemia confirmed. No secondary cause of cryofibrinogenaemia has been identified. No evidence of other vaso-occlusive disease is present.
First-Line Treatment — Insufficient Response
The previous step used corticosteroids in combination with oral anticoagulants and/or low-dose aspirin. The treatment goal — complete clinical improvement with disappearance of plasma cryofibrinogen — was not reached. This protocol represents the escalation taken after that failure.
Next Step — Partial Overview
At this stage, the approach involves the addition of immunosuppressive agents. The specific agents, selection criteria, and full sequencing are detailed in the complete protocol.
References
DOI: 10.1093/rheumatology/kew379
- Absence of cryoglobulinaemia
- No secondary cause of cryofibrinogenaemia
- No evidence of other vaso-occlusive disease
- Immunosuppressive agents should be reserved for more severe cases.
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