From Childhood to Adulthood: Investigating the Utility of Radiographic Pettersson Score in Assessing Hemophilic Arthropathy Severity

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We present findings from a prospective observational study comparing the utility of the radiographic Pettersson score (PS) and its individual radiographic components between pediatric and adult populations with hemophilic arthropathy (HA). HA, a consequence of recurrent joint bleeding, leads to synovitis and osteochondral changes, predominantly in weight-bearing joints such as the knees, elbows, and ankles.[1] The PS is a well-established radiographic tool used to assess the severity of joint damage in HA, while the Hemophilia Joint Health Score (HJHS) evaluates clinical severity and joint function.[2] [3] [4] [5] Despite the utility of the PS in assessing joint damage, there is limited comparative data on its effectiveness across pediatric and adult populations. The study also examines how these components correlate with clinical severity, as assessed by the HJHS.

The study was conducted at a tertiary care hospital over 2 years with approval from the Institute Ethics Committee and written informed consent was obtained from each patient/parents of the children included in the study. It included 27 patients (50 affected joints) with hemophilia A or B, divided into pediatric (0–18 years) and adult (> 18 years) groups. Exclusion criteria included coexisting joint diseases or previous joint surgeries. This scale evaluates eight radiographic features: osteoporosis (0/1), enlarged epiphysis (0/1), irregular subchondral surface (0/1/2), joint space narrowing (0/1/2), subchondral cysts (0/1/2), erosion of joint margin (0/1), gross incongruity (0/1/2), and joint deformity (0/1/2). The total score ranges from 0 to 13[3] ([Fig. 1]). Clinical severity was evaluated using the HJHS version 2.1, based on factors such as swelling, muscle atrophy, range of motion, strength, and pain, with scores ranging from 0 to 24, where higher scores indicate greater severity.[4] [5] [6] [7]

Fig. 1 Radiographs of knee joint (anteroposterior [AP] view) of an 18-year-old male (A) with clinical Hemophilia Joint Health Score (HJHS) score of 9 and Pettersson score of 11, illustrating: a, irregular subchondral surface, reduced joint space, and erosion of joint margin; b, subchondral cyst; c, widened intercondylar notch; d, osteoporosis. (B) Radiograph of knee joint (AP view) of a 17-year-old male with clinical HJHS score of 11 and radiographic Pettersson score of 12; a, soft tissue swelling; b, widening of epiphysis; c, subchondral cyst; d, irregular subchondral surface, erosion of joint margin, and narrowing of joint space.

Among the 27 patients, 14 were pediatric and 13 were adults. Hemophilia A was present in 23 patients (85.18%) and hemophilia B in 4 (14.8%). The most common radiographic abnormalities were joint space narrowing (80%) and erosion of the joint margin (66%), followed by osteoporosis (46%). Gross incongruence and joint deformities were rare and found only in adults. Adults had significantly higher prevalence of osteoporosis, subchondral surface irregularities, joint space narrowing, subchondral cysts, and joint margin erosion compared with pediatric patients ([Table 1]). The mean PS was significantly higher in adults (5.7 ± 2.1) compared with pediatrics (2.1 ± 2.3) (p < 0.01), and the mean HJHS score was also higher in adults (11.0 ± 4.5 vs. 8.1 ± 5.0, p = 0.039). A positive correlation between PS and HJHS scores was found in the pediatric group (R ² = 0.314), but not in the adult group (R ² = 0.044). A significant positive correlation between age and the PS was also observed (R ² = 0.45; p = 0.01). In the pediatric group, features such as enlarged epiphyses, subchondral surface irregularities, joint space narrowing, and joint margin erosion were significantly associated with higher clinical severity ([Table 2]). In contrast, osteoporosis was the only feature associated with higher clinical severity in the adult group.

Table 1 Summary of radiographic changes in 50 study joints of 27 patients along with comparison of various radiographic changes in 25 pediatric study joints and 25 adult study joints

Radiographic change	Present	Pediatric age group	Adult age group	p-Value
Osteoporosis	23 (46%)	6 (24%)	17 (68%)	0.002
Enlarged epiphysis	9 (18%)	2 (8%)	7 (28%)	0.07
Irregular subchondral surface	31 (62%)	8 (32%)	23 (92%)	< 0.01
Narrowing of joint space	40 (80%)	15 (60%)	25 (100%)	< 0.01
Subchondral cyst	20 (40%)	5 (20%)	15 (60%)	< 0.01
Erosion of joint margin	33 (66%)	9 (36%)	24 (96%)	< 0.01
Gross incongruence	2 (4%)	0 (0%)	2 (8%)	0.245
Joint deformity	1 (2%)	0 (0%)	1 (4%)	0.5

Table 2 Comparative analysis of radiographic changes in adult and pediatric patients and their comparison with the mean HJHS scores

Radiographic changes		Mean HJHS score ± SD
		Pediatric (n = 25)	Adult (n = 25)	Total (n = 50)
Osteoporosis	Present	9.0 ± 6.45 (n = 6)	12.35 ± 3.65 (n = 17)	11.48 ± 4.63 (n = 23)
	Absent	7.84 ± 4.69 (n = 19)	8.13 ± 5.17 (n = 8)	8.19 ± 4.62 (n = 27)
	p-Value	0.634	0.027	0.017
Enlarged epiphyses	Present	16.0 ± 2.82 (n = 2)	12.86 ± 4.18 (n = 7)	8.88 ± 4.66 (n = 9)
	Absent	7.44 ± 4.61 (n = 23)	10.28 ± 4.59 (n = 18)	13.56 ± 4.00 (n = 41)
	p-Value	0.018	0.210	0.008
Irregular subchondral surface	Present	11.63 ± 3.46 (n = 8)	11.00 ± 4.67 (n = 23)	11.16 ± 4.34 (n = 31)
	Absent	6.47 ± 4.88 (n = 17)	11.00 ± 4.24 (n = 2)	7.28 ± 4.84 (n = 19)
	p-Value	0.014	1.000	0.006
Narrowing of joint space	Present	10.80 ± 0.29 (n = 15)	11.00 ± 4.55 (n = 25)	10.91 ± 4.09 (n = 40)
	Absent	4.10 ± 4.58 (n = 10)	0 (n = 0)	4.44 ± 4.70 (n = 10)
	p-Value	< 0.001	NA	0.480
Erosion of joint margins	Present	12.11 ± 3.37 (n = 9)	10.88 ± 4.60 (n = 24)	11.21 ± 4.23 (n = 33)
	Absent	5.88 ± 4.44 (n = 16)	14.00 ± 0.00 (n = 1)	6.69 ± 4.67 (n = 17)
	p-Value	0.001	0.513	0.002

Abbreviations: HJHS, Hemophilia Joint Health Score; NA, not available; SD, standard deviation.

Our study reveals age-related differences in radiographic manifestations of HA. In pediatric patients, radiographic features like enlarged epiphyses, irregular subchondral surfaces, joint space narrowing, and joint margin erosion were strongly associated with clinical severity. In adults, osteoporosis was the only feature that correlated significantly with clinical severity, while joint deformities and gross incongruence showed no correlation. The higher severity in adults likely reflects prolonged disease duration and accumulated joint damage. Although the PS correlated with clinical severity in pediatric patients, this relationship was absent in adults. Inability to predict joint disease has also been indicated in previous studies.[8] [9]

In conclusion, the study reaffirms that PS is a valuable tool for assessing radiographic severity in pediatric patients. However, its utility in adults with advanced disease is limited. These findings highlight the need for alternative diagnostic methods in adults and underscore the importance of considering age-related differences in the radiographic evaluation of HA.

 

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Conflict of Interest

None declared.

• References

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Publication History

Article published online:
04 June 2025