Hemophilic arthropathy: how to diagnose subclinical bleeding early and how to orthopedically treat a damaged joint

ABSTRACT

Introduction

It is important to know the current status of hemophilic arthropathy diagnoses, treatments, complications, and outcomes in developed countries.

Areas covered

A bibliographic search in PubMed for articles published from 1 January 2019 through 12 June 2023 was performed.

Expert opinion

In developed countries with specialized hemophilia treatment centers, primary hematological prophylaxis (started before the age of 2 years and after no more than one joint bleed) has almost completely eliminated the joint-related problems of the disease. The ideal goal of zero hemarthroses can be achieved only with intense and well-dosed prophylaxis: intravenous infusion of coagulation factor – standard half-life or extended half-life; periodic or subcutaneous injections of nonfactor products (emicizumab or fitusiran). However, hemophilic arthropathy continues to occur due to subclinical joint hemorrhages. In one study, 16% of the joints without reported hemarthroses showed signs of previous subclinical bleeding (hemosiderin deposits with/without synovial hypertrophy on magnetic resonance imaging were deemed signs of previous subclinical bleeding), rendering evidence for subclinical bleeding in people with severe hemophilia with lifelong prophylaxis treatment. Subclinical joint hemorrhages can be averted only by employing accurate and tailored prophylaxis.

KEYWORDS:

• Hemophilia
• hemarthrosis
• synovitis
• arthropathy
• Diagnosis
• treatment

Article highlights

• Lifetime hematological prophylaxis must be the standard of care for all people with hemophilia worldwide.

• Hemarthrosis and synovitis, both in individuals declaring joint pain and in asymptomatic ones, must be sought by means of POCUS to unveil subclinical hemarthrosis and synovitis.

• Subclinical joint hemorrhages can be avoided only by an accurate and tailored prophylaxis.

• In acute hemarthrosis, arthrocentesis makes the recuperation faster.

• The best approach for synovitis is synovectomy. Chemical synovectomy is especially practical in developing countries where radiosynovectomy is not available, although it appears to be less beneficial than radiosynovectomy.

• When joint degeneration is severe, painful, and disabling and does not respond to conservative management (hematological prophylaxis, analgesics, COX-2 inhibitors, Physical Medicine and Rehabilitation), surgical treatment should be considered.

Declaration of interest

The author has 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 manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.