Morphea Activity Measure Responsive to Changes in Disease Activity for Localized Scleroderma

The Morphea Activity Measure (MAM) is both internally and externally responsive to shifts in disease activity, according to new findings, though additional validation using mixed cohorts of many different ages and specialties may be warranted.¹

Such findings were the result of new research—led by Maria Teresa García-Romero, MD, MPH, of the department of dermatology at the National Institute of Pediatrics in Mexico City—conducted to assess MAM’s internal and external responsiveness to shifts in pediatric individuals’ disease activity.

The investigators pointed out that MAM had been developed as a validated clinical and research tool designed to assess the various subtypes of morphea, also known as localized scleroderma.

“Evaluation of the performance of MAM in pediatric patients showed very good interrater and intrarater concordance as well as a strong correlation with mLoSSI and the Physician Global Assessment (PGA),” García-Romero and colleagues wrote. “This study sought to assess the internal and external responsiveness of MAM to changes in disease activity in pediatric patients.”²

Background and Methods

The research team involved 4 specified research sites in their research, the design of which was prospective, longitudinal, and prognostic. These 4 sites were the National Institute of Pediatrics in Mexico City, Mexico; the Hospital for Sick Children in Toronto, Ontario, Canada; Seattle Children’s Hospital in Seattle, Washington; and Wisconsin Children’s Hospital in Milwaukee.

The research was carried out from October 2021 – January 2023, and at each site consecutive subjects with morphea were recruited by the team at each of the sites. Collection of data took place at the point of baseline as well as during a follow-up interaction at least 3 months following this period.

Standard medical care was given to the participants throughout the investigators’ study, with disease activity having been evaluated through the use of PGA, mLoSSI, MAM, and PtGA. The team noted that the mLoSSI is designed to evaluate 3 elements of disease activity within 18 different regions on the body without considering size of lesions.

Both the PGA and PtGA tools utilize a 100-mm visual analog scale for their assessments. Such measurements were noted by the research team at the time of participant enrollment and in their follow-up visits.

Ethnicity was also self-reported by participants to consider the sociopolitical determinants impacting health, and subjects’ skin tones were identified through utilization of the New Immigrant Survey Skin Color Scale.

Findings

Overall, the investigators included a total of 43 individuals in their work, noting an average morphea onset age of 7.11 years and adding that 60.5% of the subjects had been female. Those demonstrating improvements in their disease activity, as determined by the PGA, showed a much greater mean change in their reported MAM scores (−18.75 [95% CI, −31.92 to −5.57]) versus individuals whose activity showed no shifts (2.73 [95% CI, −1.97 to 7.45]).

The MAM score percentage change was also described by the research team as being much higher among such individuals (−108.08% [95% CI, −155.21% to −60.95%] vs. −24.11% [95% CI, −81.22% to 32.99%]). Such trends were also noted by the team when they implemented the mLoSSI assessment, given that improved subjects had a mean change of −24.15 (95% CI, −41.89 to −6.41) and a percentage change of −172.06% (95% CI, −263.68% to −80.45%), compared to the non-changers (mean change of −1.30 [95% CI, −8.50 to 5.70]; percentage change of −21.57% [95% CI, −48.13% to 4.97%]).

The investigators also reported that the standardized response mean of the MAM tool was shown to be distinct between the cohorts for both measures, with those showing diminished activity being quite responsive in their PGA (−0.75 [95% CI, −1.29 to −0.22]) and mLoSSI (−0.97 [95% CI, −1.69 to −0.25]). Those remaining static had none to small measures of responsiveness based on the PGA (0.11 [95% CI, −0.08 to 0.30]) and mLoSSI (−0.05 [95% CI, −0.34 to 0.23]).

The research team concluded that a was a strong and substantial correlation was present between the percentage change in participants’ MAM scores and the subjects’ changes in mLoSSI (ρ = 0.69; P P P = .09).

“A limitation of this study is the multicentric design, which posed implementational and statistical challenges but was countered by training investigators and using percentage change, SES, and SRM in the analysis to limit the possibility of effect size being influenced by scale proportions,” they wrote. “Also, there was potential selection bias for patients with severe disease cared for at hospitals, with most having linear disease, which may limit generalizability.”

References

1. ^{García-Romero MT, Brandling-Bennett HA, Pope E, et al. Responsiveness to Change of the Morphea Activity Measure in Pediatric Patients. JAMA Dermatol. Published online June 05, 2024. doi:10.1001/jamadermatol.2024.1350.}
2. ^{García-Romero MT, Tollefson M, Pope E, et al. Development and validation of the Morphea Activity Measure in patients with pediatric morphea. JAMA Dermatol. 2023;159(3):299-307. doi:10.1001/jamadermatol.2022.6365.}