The Musculoskeletal Working Group on Hemophilia brought together experts to develop guidelines about "Synovitis in Hemophilia". The determination of the trough level, i.e. the factor level immediately before the next injection, and patient adherence are relevant for prophylaxis.
Experts provide advice on the treatment of chronic synovitis
The Musculoskeletal Working Group on Hemophilia brought together the experts in the field in order to develop guidelines about "Synovitis in Hemophilia" and how to successfully prevent chronic synovitis and ensure that patients receive early care. The determination of the through level, i.e. the factor level immediately before the next injection, as well as the patient adherence, is relevant for the prophylaxis necessary for prevention. The core results of the guideline "Synovitis in Hemophilia" are listed next.
Prevention of chronic synovitis: permanent treatment
In the case of severe hemophilia, and more rarely in the case of moderate hemophilia, early and sufficient prophylactic substitution therapy should be administered to prevent bleeding and its consequences.
Based on sufficient experience in the guideline group, the raising of the trough level in the context of individualized prophylactic substitution therapy in children with severe hemophilia should be carried out at ≥ 3%.
In the case of adult hemophiliacs with already arthropathic altered joints, 1-3% of the trough level in prophylactic substitution therapy ≥ should, therefore, be raised in view of the available study results.
In individual cases, it may be necessary to temporarily raise the trough level at ≥ 10% in order to improve individual pain and bleeding symptoms and to carry out physiotherapy.
Acute synovitis therapy
In the case of acute synovitis as a result of acute joint bleeding, the initial dose of the coagulation preparation should be 40-60 IU7kg 1-2 times daily, depending on the severity and extent of the bleeding. Higher individual doses may be required, especially for children.
The reduction of the dose, as well as the extension of the intervals, should depend on the clinical findings. A too rapid dose reduction and/or interval extension can promote chronic synovitis.
In the case of very pronounced joint hemorrhage with massive swelling, the puncture may be considered in individual cases.
Accompanying measures are immobilization, if necessary joke medication (no acetylsalicylic acid), anti-inflammatory therapy and physiotherapeutic treatment.
Chronic synovitis therapy
In the case of chronic synovitis, treatment with coagulation preparations is currently the most important prerequisite for preventing bleeding.
Neo-angiogenesis leads to an increased tendency to bleed. In order to prevent renewed (micro-) bleeding into the joint, the trough level of factor VIII or IX can be raised to a minimum of 30%. At the same time, a radio-synoviorthesis (medical synovectomy) should be considered.
The trough level should be checked at intervals of 7-10 days, then monthly for up to half a year. These controls should also include a clinical examination to investigate overheating, capsule thickening, impaired mobility, or muscular atrophy.
If the synovitis has not improved sufficiently, either another half year of prophylactic substitution therapy with the same intensity can be performed or a new radiosynoviorthesis or synovectomy can be considered.
In addition to the substitution therapy of FVIII or FIX, the following additional measures should be carried out:
Physiotherapeutic accompanying treatment to avoid or improve muscular atrophy that has already occurred as well as coordination and gait disorders.
In addition to the clinical examination, controls should be performed by sonography every 7-10 at the beginning, monthly thereafter and every 3-6 months with contrast media using MRI. For small children, the very time-consuming and stressful MRI examination (sedation) must be carefully weighed.
"Dr. Hans-Hermann Brackmann from the Hemophilia Centre at the University of Bonn appeals to the participants of the session: "Synovitis must be treated at an early stage. The aim of prophylactic treatment of hemophilia must be to prevent the development of chronic synovitis. With the extended half-lives of factor preparations and the associated increase in patient adherence through fewer injections with the same trough levels, there is now hope for successful prevention.
Brackmann, H.H. (Berlin). MIS18-5 Synovitis from the point of view of an experienced hemostaseology. IN: 10:00 - 11:30 MIS 18 Musculoskeletal Working Group Hemophilia. What did I learn from the guideline "Synovitis in Hemophilia"? 63rd Annual Meeting of the Society of Thrombosis and Haemostasis Research, Berlin, Germany. 27 Feb. - 2 Mar. 2019.