Synovitis in hemophilia: preventing, detecting, and treating joint bleeds

ABSTRACT

Introduction

Most bleeding events in individuals with hemophilia occur within the ankle, knee, and elbow joints. Should the bleeding persist, the synovial membrane starts to hypertrophy and a vicious cycle of chronic hemophilic synovitis (CHS) occurs, leading to joint destruction.

Areas covered

This article covers the prompt diagnosis of CHS by point-of-care ultrasonography (POC-US) and its treatment by means of several types of synovectomy.

Expert opinion

It is essential to prevent, detect and treat hemophilic synovitis, because it indicates that the joint has bled and is at risk of bleeding further. Prophylaxis with standard half life (SHL) factor VIII (FVIII) concentrate is the standard of care for individuals with severe hemophilia A and can also be considered for selected patients with moderate disease. Several years of real-world experience with extended half life (EHL) FVIII, emicizumab, and other drugs in development will be needed to ascertain their final effect on bleeding and its complications. We must look for synovitis in individuals declaring joint pain and in asymptomatic patients, and POC-US is the most reasonable imaging instrument with which to carry out periodic joint screening. Radiosynovectomy, chemical synovectomy, and arthroscopic synovectomy markedly reduce bleeding events.

KEYWORDS:

• Hemophilia
• synovitis
• prevention
• diagnosis
• treatment
• radiosynovectomy
• chemical synovectomy
• arthroscopic synovectomy

Article highlights

• It is essential to prevent, detect and treat CHS, because it indicates that the joint has bled and is at risk of bleeding further.

• Prophylaxis with SHL FVIII concentrates is the standard of care for individuals with severe hemophilia A and might also be considered for selected patients with moderate disease. Several years of real-world experience with EHL FVIII, emicizumab, and other drugs in development will be needed to ascertain their effect in the restraint of bleeding and its complications.

• We must assess CHS in individuals declaring joint pain and in asymptomatic patients, with POC-US the most reasonable imaging instrument with which to carry out periodic joint screening.

• Without POC-US, the capability to recognize subclinical CHS is highly diminished, mainly in the elbows.

• Radiosynovectomy, chemical synovectomy, and arthroscopic synovectomy markedly abate bleeding events.

• Chemical synovectomy (rifampicin and oxytetracycline) appear to be less beneficial than with RS, but it is especially practical in developing countries where RS is not available.

• Rifampicin synovectomy appears to be more effective in small joints (elbows and ankles), than in bigger ones (knees).

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this man [1]uscript have no relevant fi [2]nancial or other relationships to disclose.

Additional information

Funding

This paper was not funded.