Background/Purpose:

Lower extremity deep vein thrombosis (LEDVT) is a disabling complication of Behcet’s syndrome (BS). Relapses are frequent and cause permanent disability due to post-thrombotic syndrome (1). The management of LEDVT in Behcet’s syndrome (BS) constitutes mainly of azathioprine (AZA) and corticosteroids (CS) as first-line agents (2). Interferon-α (IFN) has been used with good results in the management of eye involvement of BS. However, data regarding its efficacy for vascular involvement has been scarce (3). We aimed to evaluate the efficacy and safety of IFN for LEDVT in BS.

Methods:

All BS pts who had a first episode of acute LEDVT since March 2010 are being prospectively followed with a standard protocol in our dedicated BS center. Acute LEDVT is confirmed by Doppler ultrasonography (DUS) at initial diagnosis and serial DUS assessment is performed and also repeated in case of clinical suspicion of relapse. Our standard treatment strategy consists of AZA and CSs in pts with LEDVT. IFN has been used in pts who were refractory or intolerant to this regimen, or who had co-existing eye involvement. Our endpoints for assessing the efficacy of IFN have been recanalization of the index thrombus and prevention of relapses. Recanalization has been assessed in the transverse plane and defined as the ratio of the vein area at maximum compressibility to the non-compressed vein area. Good recanalization was defined as a ratio of at least 50%. Adverse events during IFN use were recorded.

Results:

33 pts with LEDVT (26 M/7 F) were prospectively followed for a mean of 40.7±13.4 mo. Among these IFN was started in 17/33 for mainly vascular involvement. In 2 pts IFN was started at the first episode of LEDVT due to co-existing uveitis. Seven pts were treated with IFN due to LEDVT relapses under AZA. In the remaining 9 pts, the reasons for switching from AZA to IFN were adverse events with AZA (n=2), relapse of superficial thrombophlebitis (n=4), leg ulcers due to severe post-thrombotic syndrome (n=2) and eye involvement (n=1). Among 17 pts treated with IFN during a mean follow-up of 29±20 mo, 3 pts already had good recanalization when starting IFN. In the remaining 14 pts, 13 (93%) had good recanalization under IFN. Two pts (11%) experienced relapses. One of the 2 pts who had a relapse had had poor recanalization despite IFN. In contrast, among the 29 pts treated with AZA with a mean follow-up of 20.2±15.8 mo, only 13 (45%) had good recanalization. 13 (45%) pts experienced relapses under AZA treatment and 9 (69%) of those pts had poor recanalization. Overall we observed 23 LEDVT relapses in 15 pts. Relapse rates were 29%, 37% and 45% at 6, 12 and 24 mo respectively. The only adverse event with IFN causing drug withdrawal was thyroiditis in 1 patient.

Conclusion:

The relapse rate for LEDVT in BS is high despite AZA treatment. IFN seems to be a promising agent for preventing LEDVT relapses and achieving good recanalization, an important predictor of relapse. The small number of pts and the lack of a parallel control group are the limitations of this prospective study.

References:

1) Melikoglu M. Arthritis Rheumatol 2014

2) Alibaz-Oner F. Medicine (Baltimore) 2015

3)Calguneri M. Ann Rheum Dis. 2003

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/interferon-alpha-for-the-management-of-lower-extremity-deep-vein-thrombosis-in-behcets-syndrome-a-case-series/