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Haemophilia A and B - evaluation of the Swedish prophylactic regimen by magnetic resonance imaging

Lundin, Björn LU ; Baghaei, Fariba ; Holmström, Margareta ; Petrini, Pia ; Müller, Gunilla LU ; Månsson, Sven LU orcid and Ljung, Rolf LU orcid (2023) In Haemophilia 29(1). p.193-198
Abstract

INTRODUCTION: Sweden has been a pioneer in the prophylactic treatment of haemophilia. Magnetic resonance imaging (MRI) can detect small changes in joints and can therefore give an indication of a risk of developing arthropathy.

AIM: To use MRI to evaluate the outcome of the Swedish 'high-dose regimen' and correlate the findings to age, bleeds, joint score and physical activity.

METHODS: The study group comprised 48 Swedish male patients, mean age 25 years (range 12-33 years), with severe or moderate haemophilia A or B. Data on the Haemophilia Joint Health Score (HJHS) were available and physical activity was evaluated by a self-reported questionnaire.

RESULTS: MRI score was recorded in 188 joints. Twenty out of 48... (More)

INTRODUCTION: Sweden has been a pioneer in the prophylactic treatment of haemophilia. Magnetic resonance imaging (MRI) can detect small changes in joints and can therefore give an indication of a risk of developing arthropathy.

AIM: To use MRI to evaluate the outcome of the Swedish 'high-dose regimen' and correlate the findings to age, bleeds, joint score and physical activity.

METHODS: The study group comprised 48 Swedish male patients, mean age 25 years (range 12-33 years), with severe or moderate haemophilia A or B. Data on the Haemophilia Joint Health Score (HJHS) were available and physical activity was evaluated by a self-reported questionnaire.

RESULTS: MRI score was recorded in 188 joints. Twenty out of 48 patients had a score of ≥1 (range 1-13) in 31 joints of which 3/31 scores were in the knees and 28/31 in the ankles. No correlation was found between the number of recorded bleeds and the MRI score or between HJHS and MRI score. There was no correlation between the physical activity and the number of joint bleeds per se, but a trend (OR 3.0) that those most physically active (19/48; 39.6%), more frequently had an MRI score of ≥1 with an overweight for the right ankle.

CONCLUSION: The Swedish prophylactic model offers protection against haemophilia joint arthropathy but will still not prevent osteochondral changes in some patients at young age. MRI of the ankles can signal risk of future arthropathy and indicate need to modify the prophylactic regimen.

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author
; ; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Haemophilia
volume
29
issue
1
pages
193 - 198
publisher
Wiley-Blackwell
external identifiers
  • scopus:85143975826
  • pmid:36469433
ISSN
1351-8216
DOI
10.1111/hae.14693
language
English
LU publication?
yes
additional info
© 2022 The Authors. Haemophilia published by John Wiley & Sons Ltd.
id
6f29697a-862f-4172-a854-83dab6121ee9
date added to LUP
2022-12-12 12:06:17
date last changed
2024-09-07 09:17:46
@article{6f29697a-862f-4172-a854-83dab6121ee9,
  abstract     = {{<p>INTRODUCTION: Sweden has been a pioneer in the prophylactic treatment of haemophilia. Magnetic resonance imaging (MRI) can detect small changes in joints and can therefore give an indication of a risk of developing arthropathy.</p><p>AIM: To use MRI to evaluate the outcome of the Swedish 'high-dose regimen' and correlate the findings to age, bleeds, joint score and physical activity.</p><p>METHODS: The study group comprised 48 Swedish male patients, mean age 25 years (range 12-33 years), with severe or moderate haemophilia A or B. Data on the Haemophilia Joint Health Score (HJHS) were available and physical activity was evaluated by a self-reported questionnaire.</p><p>RESULTS: MRI score was recorded in 188 joints. Twenty out of 48 patients had a score of ≥1 (range 1-13) in 31 joints of which 3/31 scores were in the knees and 28/31 in the ankles. No correlation was found between the number of recorded bleeds and the MRI score or between HJHS and MRI score. There was no correlation between the physical activity and the number of joint bleeds per se, but a trend (OR 3.0) that those most physically active (19/48; 39.6%), more frequently had an MRI score of ≥1 with an overweight for the right ankle.</p><p>CONCLUSION: The Swedish prophylactic model offers protection against haemophilia joint arthropathy but will still not prevent osteochondral changes in some patients at young age. MRI of the ankles can signal risk of future arthropathy and indicate need to modify the prophylactic regimen.</p>}},
  author       = {{Lundin, Björn and Baghaei, Fariba and Holmström, Margareta and Petrini, Pia and Müller, Gunilla and Månsson, Sven and Ljung, Rolf}},
  issn         = {{1351-8216}},
  language     = {{eng}},
  number       = {{1}},
  pages        = {{193--198}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Haemophilia}},
  title        = {{Haemophilia A and B - evaluation of the Swedish prophylactic regimen by magnetic resonance imaging}},
  url          = {{http://dx.doi.org/10.1111/hae.14693}},
  doi          = {{10.1111/hae.14693}},
  volume       = {{29}},
  year         = {{2023}},
}