Background/Purpose

Familial Mediterranean fever (FMF) is a hereditary and an auto-inflammatory disease predominantly characterized by repeated attacks of fever, abdominal pain, pleuritic chest pain, arthritis and erysipelas-like erythema.The MEditerranean FeVer (MEFV) gene mutations altering the structure and function of pyrin protein play a significant role in the pathophysiology of the disease. Mutated pyrin is associated with the loss of delicate control of the inflammatory pathways, which results in a prolonged or augmented inflammation that predisposes these patients to a pro-inflammatory state. This increased inflammation might lead to susceptibility to vascular comorbidities in FMF patients.  The aim of this study was to assess the effects of this increased inflammation on cerebral blood flow velocity with transcranial Doppler (TCD) ultrasonography.

Methods In this study, 30 subjects were enrolled for FMF and healthy control groups. All patients with FMF were under colchicine treatment and they were in an attack free period. Bilateral middle cerebral artery (MCA) peak-systolic, end-diastolic, and mean blood flow velocities; Gosling’s pulsatility index values; and Pourcelot’s resistance index values were recorded and compared with each other.  

Results

There were 30 subjects in each group. Men/women ratio and mean age in FMF and control groups were 4/3, 26/27 and 34,7±5,9 vs.32,3±4,7 respectively. Peak-systolic, end-diastolic, and mean blood flow velocities of bilateral MCA were  significantly higher in FMF group when compared with the control group (Table 1).  

Table 1 Transcranial Doppler data of FMF group compared with control group. 

	FMF group (mean±SD)	Control group (mean±SD)	p-value
L-peak-systolic BFV	143,3± 19,5	104,5± 13	<0,001
L-end-diastolic BFV	56,4± 13,5	38,4± 6,2	<0,001
L-mean BFV	90,9± 13,8	61,8±  8,2	<0,001
L-PI	0,85  ± 0,07	0,9±0 ,09	0,02
L-RI	0,59± 0 ,03	0,6±0,05	0,57
R-peak-systolic BFV	145,2± 22,3	103,5± 17,05	<0,001
R-end-diastolic BFV	59,5±13	36,7± 12,36	<0,001
R-mean BFV	90,7±15,47	63,2± 10,5	<0,001
R-PI	0,8±0,08	0,85±0 ,09	0,89
R-RI	0,56± 0,04	0,63±0 ,06	0,002

L, left; R, right; PI, pulsatility index; RI, resistance index; BFV; blood flow velocity.

 Conclusion

There has been recently considerable attention concerning the possible causal role of systemic inflammation in the development of atherosclerosis in patients with rheumatic diseases. The attacks of FMF with clinical inflammation is only the tip of the iceberg, inflammation maintains in attack-free remission periods in 30% of patients with FMF. This maintaining subclinical inflammation induces endothelial dysfunction, and increases the risk of developing significant complications such as atherothrombosis, anemia, splenomegaly, decreased bone mineral density, heart disease, and life-threatening secondary systemic amyloidosis. In this study, we investigated the effects of clinical and subclinical inflammation regarding FMF disease on cerebral blood flow parameters. In our study, the mechanisms that underlie significantly increased blood flow velocities of  the FMF group is most likely to be due to mild diffuse subclinical atherosclerosis. Consequently, our results suggest that persistent clinical and subclinical inflammation in FMF patients  causes increased cerebral blood flow velocities.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/changes-in-cerebral-blood-flow-velocity-in-patients-with-familial-mediterranean-fever/