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Evidence for circulating fibrin in familial Mediterranean fever
Abstract:
Cryofibrinogenemia was found in 10 of 24 plasma samples (42%) from subjects with FMF. This precipitate was found during active disease as well as during intervals between crises. We found a higher incidence of cryofibrinogenemia in subjects with mild to moderately severe disease not being treated with colchicine (six of eight) as compared with colchicine-treated subjects who were in partial or complete clinical remission (four or 16; p less than 0.02). All cryofibrinogen precipitates contained fibrin, as assessed by electrophoretic analyses showing the presence of multimeric crosslinked forms of fibrin(ogen) linked by gamma-dimers. This finding in clinical specimens supports the hypothesis that fibrin in an obligatory component of cryofibrinogen. Fibrin was also found in HPF (two of six specimens) prepared from cryofibrinogen-negative FMF plasmas, thus showing that soluble forms of fibrin are even more prevalent in this disorder than is indicated by the frequent finding of cryofibrinogenemia.
Insights
Cryofibrinogenemia, a plasma precipitate containing fibrin, is common in Familial Mediterranean Fever (FMF). Its presence may correlate with disease severity and colchicine treatment status in FMF patients.
Area of Science:
- Hematology
- Immunology
- Genetics
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- Cryofibrinogenemia is characterized by the precipitation of cryofibrinogen from plasma at low temperatures.
- The exact role of cryofibrinogenemia in FMF pathogenesis remains unclear.
Purpose of the Study:
- To investigate the incidence and characteristics of cryofibrinogenemia in patients with FMF.
- To explore the association between cryofibrinogenemia and clinical parameters in FMF, including disease activity and colchicine treatment.
Main Methods:
- Plasma samples from 24 FMF subjects were analyzed for cryofibrinogenemia.
- Electrophoretic analyses were performed on cryofibrinogen precipitates to identify their composition.
- High-temperature precipitation (HPF) was used to detect soluble fibrin in cryofibrinogen-negative samples.
Main Results:
- Cryofibrinogenemia was detected in 42% (10/24) of FMF plasma samples, occurring during both active disease and remission periods.
- Cryofibrinogen precipitates consistently contained fibrin, confirmed by electrophoretic detection of crosslinked fibrin(ogen) forms.
- A higher incidence of cryofibrinogenemia was observed in untreated patients with mild to moderate FMF compared to colchicine-treated patients in remission (p < 0.02).
- Soluble fibrin, detected by HPF, was present in cryofibrinogen-negative FMF plasmas, suggesting even greater prevalence of fibrin in FMF.
Conclusions:
- Fibrin is an integral component of cryofibrinogen in FMF patients.
- Cryofibrinogenemia is a frequent finding in FMF and may be associated with disease activity and lack of colchicine treatment.
- The presence of soluble fibrin in FMF plasma is more widespread than indicated by cryofibrinogenemia alone, suggesting a potential role in FMF pathophysiology.