TY - JOUR
T1 - Arthropathy on X-rays in 363 persons with hemophilia
T2 - long-term development, and impact of birth cohort and inhibitor status
AU - van Heel, Dayna A M
AU - Foppen, Wouter
AU - Fischer, Kathelijn
N1 - Publisher Copyright:
© 2024 The Authors
PY - 2024/2
Y1 - 2024/2
N2 - BACKGROUND: Arthropathy following repeated bleeding is common in persons with hemophilia. Since the introduction of prophylaxis, treatment has intensified and joint health has improved. However, data on the long-term development of arthropathy are still scant.OBJECTIVES: To evaluate long-term arthropathy development since the introduction of prophylaxis according to birth cohort, hemophilia severity, and inhibitor status.METHODS: This single-center historic cohort study included persons with severe and moderate hemophilia A and hemophilia B born between 1935 and 2005. Arthropathy on X-rays was evaluated using the Pettersson score. Patient and joint characteristics were studied per birth cohort (<1970, 1970-1980, 1981-1990, and >1990) and compared according to hemophilia severity. The distribution of affected joints and cumulative incidence of arthropathy were analyzed. The association of Pettersson score with birth cohort and inhibitor characteristics was explored using multivariable regression analyses adjusted for age at evaluation.RESULTS: In total, 1064 X-rays of 363 patients were analyzed. Of persons with severe hemophilia (n = 317, 87.3%), 244 (77.0%) developed arthropathy. Prophylaxis was started at younger ages over time, from a median of 18 to 2.1 years, and concomitantly, arthropathy decreased in consecutive birth cohorts. Ankles were most commonly affected in 188 of 258 (72.9%) patients. Persons with moderate hemophilia (n = 46, 12.7%) had a lower risk of arthropathy (27/46 [58.7%]), but a reduction over time was less pronounced. In the multivariable analyses, birth cohort and age at evaluation were predictors for the development of arthropathy, while inhibitor status showed no association.CONCLUSION: The development and severity of arthropathy have decreased over the past decades. However, patients have remained at risk for developing arthropathy, especially in their ankles.
AB - BACKGROUND: Arthropathy following repeated bleeding is common in persons with hemophilia. Since the introduction of prophylaxis, treatment has intensified and joint health has improved. However, data on the long-term development of arthropathy are still scant.OBJECTIVES: To evaluate long-term arthropathy development since the introduction of prophylaxis according to birth cohort, hemophilia severity, and inhibitor status.METHODS: This single-center historic cohort study included persons with severe and moderate hemophilia A and hemophilia B born between 1935 and 2005. Arthropathy on X-rays was evaluated using the Pettersson score. Patient and joint characteristics were studied per birth cohort (<1970, 1970-1980, 1981-1990, and >1990) and compared according to hemophilia severity. The distribution of affected joints and cumulative incidence of arthropathy were analyzed. The association of Pettersson score with birth cohort and inhibitor characteristics was explored using multivariable regression analyses adjusted for age at evaluation.RESULTS: In total, 1064 X-rays of 363 patients were analyzed. Of persons with severe hemophilia (n = 317, 87.3%), 244 (77.0%) developed arthropathy. Prophylaxis was started at younger ages over time, from a median of 18 to 2.1 years, and concomitantly, arthropathy decreased in consecutive birth cohorts. Ankles were most commonly affected in 188 of 258 (72.9%) patients. Persons with moderate hemophilia (n = 46, 12.7%) had a lower risk of arthropathy (27/46 [58.7%]), but a reduction over time was less pronounced. In the multivariable analyses, birth cohort and age at evaluation were predictors for the development of arthropathy, while inhibitor status showed no association.CONCLUSION: The development and severity of arthropathy have decreased over the past decades. However, patients have remained at risk for developing arthropathy, especially in their ankles.
KW - X-rays
KW - birth cohort
KW - cohort studies
KW - hemophilia A
KW - hemophilia B
KW - joint diseases
UR - http://www.scopus.com/inward/record.url?scp=85187374690&partnerID=8YFLogxK
U2 - 10.1016/j.rpth.2024.102355
DO - 10.1016/j.rpth.2024.102355
M3 - Article
C2 - 38496709
SN - 2475-0379
VL - 8
JO - Research and practice in thrombosis and haemostasis
JF - Research and practice in thrombosis and haemostasis
IS - 2
M1 - 102355
ER -