Joint Inflammation Can Lessen Life Quality in People with Severe Hemophilia, Study Says

Chronic joint inflammation lowers the health-related quality of life in people with severe hemophilia, a European study reports, and urges physicians to be aware of this association in treating patients.

The research, “The impact of severe haemophilia and the presence of target joints on health-related quality-of-life,” appeared in the journal Health and Quality of Life Outcomes.

Nearly 80% of bleeding episodes in hemophilia are within the joints, mainly in the knees, elbows, and ankles. Frequent bleed events in the same joint lead to chronic inflammation, and reduced flexion and mobility due to fluid accumulation.

The introduction of prophylactic (preventive) treatment with clotting factor concentrates has greatly eased joint damage in patients age 30 or younger, relative to previous generations.

But the development of inhibitors against these concentrates, and the lack of effective treatments to limit their impact, can lead to greater bleeding frequency and accelerate joint damage in patients.

The psychological burden of hemophilia has historically been overlooked. As treatment advances extend life expectancy and improve patient  outcomes, recent studies have begun to analyze health-related quality of life (HRQOL).

Still, the impact of musculoskeletal complications – those including the joints, muscles, tendons, or bones – have not received much attention here.

The researchers explored factors influencing HRQOL in a large group of patients with severe hemophilia who live in France, Germany, Italy, Spain, and the U.K.

They focused on the association between target joints and HRQOL, and on how long-term clinical outcomes impact patient-reported health and well-being. Target joint status was defined as chronic inflammation in the synovium, a specialized connective tissue forming the inner surface of joints.

The scientists retrieved data from 1,285 adult patients with severe, inherited hemophilia A and B, who participated in the CHESS study, an assessment of hemophilia’s economic burden.

A total of 515 of these patients completed EQ-5D, a general tool to assess HRQOL. EQ-5D covers such factors  as mobility, self-care, everyday activities, pain/discomfort, and anxiety/depression.

A total of 692 target (chronic inflammation) joints were reported across the patient group. Compared to those with no target joints, patients with one or more target joints had significantly lower (worse) results on EQ-5D.

Data analyzed also demonstrated an association between the presence of target joints and pain and discomfort. More than eight times as many patients reported extreme pain or discomfort when a target joint was recorded.

“Our analysis demonstrates that haemophilia-related medical complications, such as the presence of one or more target joints [chronic synovitis], can have a major impact on HRQOL for persons with severe haemophilia,” the scientists wrote.

“Prevention, early diagnosis and treatment of target joints should be an important consideration for clinicians and patients when managing haemophilia,” they added.

They recommend that future studies evaluate the cost effectiveness of current therapies for severe hemophilia, combining analyses of their cost with their impact on health-related quality of life.