Use of Power Doppler Ultrasound in Haemophilia Could Predict Early Relapse of Intra Articular Bleeding after Haemarthrosis: A Prospective Cohort of 27 Haemarthrosis Monitored By Articular Ultrasound

Introduction:

Whereas prophylactic treatment with clotting factor has demonstrated superiority to prevent joint disease versus on-demand therapy in haemophilia, haemophilic arthropathy remains an important complication of the disease. Repetitive intra articular bleeding are directly correlated to this progressive joint destruction. 
In rheumatoid arthritis, the use of articular Ultrasound with Power Doppler (USPD) has demonstrated superiority to predict joint inflammation and destruction over clinical examination and biological tests. Intensity of PD ultrasound is correlate to tissue vascularisation. As hypervascularization of synovial membrane would be probably associated to occurrence of intra articular bleeding, we proposed to evaluate PD ultrasound of synovial membrane in haemophilia. after a joint haemorrhage to predict haemarthrosis relapse.

Method:

Patients with severe haemophilia A(HA), B (HB) or type 3 von Willebrand (vWD) disease with acute haemarthrosis were prospectively included in a monocentric study, from April 2013 and November 2014.
All included patients were treated using complementary daily clotting factor substitution according to clinical context.
Clinical and USPD examination of the bleeding joint were performed at diagnosis (week 0: W0), week 1 (W1), week 2 (W2), month 1 (M1), month 2 (M2) and month 6 (M6).
Physical examination considered pain, swelling and joint mobility.
USPD was performed by the same radiologist, experienced in musculoskeletal ultrasound, using General Electric LogiqE9 device, with a high frequency (6-15 MHz) linear probe (PRF = 0,8 MHz), considering joint effusion, synovial thickening, presence of Power Doppler signal and bone erosion.

Results :

21 patients were included: 16 children and 5 adults (median of age: 11,4 years-old). 14 patients presented HA, 3 HB and 4 vWD. 6 of them had inhibitors against clotting factor (4 with HA and 2 with vWD). All patients were on prophylactic regimen.
27 haemarthrosis were evaluated: 17 elbows, 7 knees and 3 ankles.
5 relapses were observed (median of time: 53 days). 3 of them had inhibitors (3/5).
Clinically, all patients had no more pain at W2, even in case of relapse. At W2, swelling joint and joint mobility were identical to those before hemarthrosis.
USPD showed:
- 20/22 joints without Power Doppler signal did not rebleed,
- all rebleeding joints presented Power Doppler signal (5/5),
- in patients with no relapse, Power Doppler signal mainly disappeared between W2 (8/16) and M1 (11/16),
- at M6, 8/12 patients presented abnormal USPD finding (6/12 joint effusion, 8/12 synovial thickening) without clinical evidence of relapse.

Conclusion:

As yet demonstrated in rheumatoid arthritis, USPD seems more sensitive than physical examination and very useful to manage haemarthrosis.
Without PD signal, the risk of early relapse seems to be very low. Moreover the presence of PD signal seems to be correlated with higher risk of relapse.
These findings would lead to better support patients with PD signal in order to avoid relapse, especially from W2 until PD signal disappeared. Persistent PD signal could justify an intensification of the treatment.
Further studies should be conducted to confirm these data.