--- title: "Atebrioz (zilurgisertib) in Fibrodysplasia ossificans progressiva: cost-effectiveness analyses and utility weights" description: "Published cost-effectiveness analyses and health-state utility weights for Atebrioz (zilurgisertib, INCB000928) in Fibrodysplasia ossificans progressiva, each value quoted from the article: 2 cost-effectiveness analyses and 1 study reporting utility weights. Analyses: Deflazacort versus prednisone in Duchenne muscular dystrophy, United States: ICER (2019); Asfotase alfa versus best supportive care in pediatric-onset hypophosphatasia, Canada: CADTH (2017). Utility weights: Patient and caregiver utility weights by functional score, ambulatory status, and ventilation status, Duchenne muscular dystrophy: Landfeldt et al. (2017). Every value is a verbatim quote from the primary article it is shown with." canonical: "https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/cost-effectiveness-analyses" published: "2026-10-22T07:49:06-04:00" modified: "2026-10-22T07:49:06-04:00" --- # Atebrioz (zilurgisertib) in Fibrodysplasia ossificans progressiva: cost-effectiveness analyses and utility weights Published cost-effectiveness analyses and health-state utility weights that a model of this product could cite, adapt, or compare against, grouped by how each article relates to the product and its indication. Every value is quoted from the article it cites, and section 3 lists the searches that found the articles. ## Overview | Item | Detail | | --- | --- | | Literature searched | Tufts Medical Center Cost-Effectiveness Analysis Registry, searched October 21, 2026; the queries are listed in section 3. | | Cost-effectiveness analyses | 2 | | Studies reporting utility weights | 1 | ## 1 Cost-effectiveness analyses ### 1.1 Analyses of Atebrioz (zilurgisertib and INCB000928) No evidence found. ### 1.2 Treatments for the same indication No evidence found. ### 1.3 The same disease in other populations or settings No evidence found. ### 1.4 Related diseases and analogous treatments #### Deflazacort versus prednisone in Duchenne muscular dystrophy, United States: ICER (2019) Relevance: Evaluates deflazacort against prednisone in Duchenne muscular dystrophy in the United States with lifetime health states defined by ambulatory status, a structure a model of fibrodysplasia ossificans progressiva could adapt for the progression to wheelchair use described in section 2.2.1.3. > “A five-state partitioned survival model was developed for this evaluation, informed by key clinical trials, cohort studies, and prior relevant studies related to economic modeling in DMD.24,36-40 The five health states were early ambulatory, late ambulatory, early non-ambulatory, late non- ambulatory, and death. Children entered the model in the early ambulatory health state at age five.” > “The modeled population consisted of children diagnosed with DMD, with diagnosis and treatment commencing at age five. All comparative treatments were assumed to be given over a lifetime in accordance with the ICER Reference Case.” > “Costs and outcomes were discounted at 3% per year.” > “In this corrected version posted on April 22, 2022, we updated the cost-effectiveness analysis to correct an error in how treatment costs were estimated for prednisone and deflazacort.” > “Deflazacort Compared to Prednisone Health Sector Perspective $663,000 $632,000 $458,000 Deflazacort Compared to Prednisone Modified Societal Perspective $692,000 $659,000 $478,000” > “The base-case results for incremental cost-effectiveness ratios comparing deflazacort to prednisone represent cost-utility results that are well beyond the range of commonly accepted thresholds of $50,000 to $150,000 per QALY despite highly favorable assumptions about treatment effects being incorporated in the model (see Table ES5).” > “Results of the probabilistic sensitivity analysis showed that at a $150,000 per QALY threshold, deflazacort had less than a 0.01% probability of being cost-effective. When increasing the threshold to $500,000 per QALY, deflazacort had roughly a 13% probability of being cost-effective.” Source: [ICER (2019)](https://icer.org/wp-content/uploads/2020/10/Corrected_ICER_DMD-Final-Report_042222.pdf) #### Asfotase alfa versus best supportive care in pediatric-onset hypophosphatasia, Canada: CADTH (2017) Relevance: Evaluates asfotase alfa against best supportive care in pediatric-onset hypophosphatasia from the Canadian health care payer perspective over a lifetime, a government review of a chronic therapy for an ultra-rare genetic bone disorder in the same jurisdiction as the Canadian palovarotene review in section 4.1. > “The reference case time horizon was lifetime (101 years), considering the Canadian publicly funded health care system perspective.” > “The economic submission was based on a Markov model with four key health states defined primarily by the severity level of disease.” > “Incremental cost of asfotase alfa versus BSC: $27,728,012” > “Incremental benefit of asfotase alfa versus BSC: 10.27 QALYs” > “CDR calculated an ICUR of $2,698,950 per QALY from the manufacturer’s results.” > “A reanalysis of the limitations surrounding the future cost of asfotase alfa (i.e., no reduction in price) and accounting for the cost of wastage of partially used vials resulted in an ICUR of $4,080,555 per QALY.” > “Considering the latter as the CDR reference case, altering the utility weights based on the extreme values defined by the confidence intervals results in ICURs of $4,018,632 and $8,828,056 per QALY, when lower and higher values are used, respectively.” Source: [CADTH (2017)](https://www.cda-amc.ca/sites/default/files/cdr/pharmacoeconomic/SR0443_Strensiq_PE_Report.pdf) ## 2 Utility weights ### 2.1 Utility weights from analyses of Atebrioz (zilurgisertib and INCB000928) No evidence found. ### 2.2 Utility weights for the same indication No evidence found. ### 2.3 Utility weights for the same disease in other populations or settings No evidence found. ### 2.4 Utility weights for related diseases and analogous treatments #### Patient and caregiver utility weights by functional score, ambulatory status, and ventilation status, Duchenne muscular dystrophy: Landfeldt et al. (2017) Relevance: Patient and caregiver utilities by ambulatory status in Duchenne muscular dystrophy, health states that correspond to the progression to wheelchair use and lifelong dependence on assistance described for fibrodysplasia ossificans progressiva in section 2.2.1.3. > “Patient utilities (ranging from 0 = dead to 1 = perfect health) were obtained from the Health Utilities Index Questionnaire and caregiver utilities from the EuroQol EQ-5D-3L” Source: [Landfeldt et al. (2017)](https://pmc.ncbi.nlm.nih.gov/articles/PMC5253157/) | Health state | Patient | Caregiver | | --- | --- | --- | | “Initial score” | “0.879 (0.037)” | “0.862 (0.016)” | | “Per lost score (multiplier)” | “0.905 (1.003)” | “0.995 (1.001)” | | “Early ambulatory” | “0.699 (0.036)” | “0.858 (0.017)” | | “Late ambulatory” | “0.607 (0.029)” | “0.839 (0.017)” | | “Early non-ambulatory” | “0.224 (0.014)” | “0.784 (0.021)” | | “Late non-ambulatory” | “0.146 (0.010)” | “0.810 (0.018)” | | “None” | “0.518 (0.027)” | “0.837 (0.014)” | | “Night-time” | “0.129 (0.017)” | “0.775 (0.030)” | | “Day- and night-time” | “0.051 (0.010)” | “0.774 (0.033)” | Source: [Landfeldt et al. (2017)](https://pmc.ncbi.nlm.nih.gov/articles/PMC5253157/) ## 3 Search strategy | Dataset | Query | Results | | --- | --- | --- | | Ratios | zilurgisertib OR Atebrioz OR "INCB000928" OR palovarotene OR Sohonos OR garetosmab | 0 | | Ratios | "fibrodysplasia ossificans progressiva" OR "heterotopic ossification" | 0 | | Weights | "fibrodysplasia" OR "heterotopic ossification" OR palovarotene OR "ossification" | 0 | | Methods | fibrodysplasia OR palovarotene OR "heterotopic ossification" OR "ALK2" | 0 | | Ratios | achondroplasia OR vosoritide OR "osteogenesis imperfecta" OR hypophosphatasia OR "asfotase" OR "X-linked hypophosphatemia" OR burosumab | 2 | | Weights | achondroplasia OR "osteogenesis imperfecta" OR hypophosphatasia OR "hypophosphatemia" OR "skeletal dysplasia" | 8 | | Weights | "Duchenne" AND (ambulatory OR wheelchair OR ambulation) | 24 | | Methods | zilurgisertib OR Atebrioz OR garetosmab OR Sohonos | 0 | ## Related pages - [Atebrioz (zilurgisertib): Fibrodysplasia ossificans progressiva](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva) · [Markdown](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva.md) - [Atebrioz (zilurgisertib) in Fibrodysplasia ossificans progressiva: Terms and abbreviations](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/terms-and-abbreviations) · [Markdown](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/terms-and-abbreviations.md) - [Atebrioz (zilurgisertib): Fibrodysplasia ossificans progressiva](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/executive-summary) · [Markdown](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/executive-summary.md) - [Atebrioz (zilurgisertib) in Fibrodysplasia ossificans progressiva: Product information and disease description](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/product-information-and-disease-description) · [Markdown](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/product-information-and-disease-description.md) - [Atebrioz (zilurgisertib) in Fibrodysplasia ossificans progressiva: Clinical evidence](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/clinical-evidence) · [Markdown](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/clinical-evidence.md) - [Atebrioz (zilurgisertib) in Fibrodysplasia ossificans progressiva: Economic information and modeling report](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/economics) · [Markdown](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/economics.md) - [Atebrioz (zilurgisertib) in Fibrodysplasia ossificans progressiva: References](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/references) · [Markdown](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/references.md) - [Atebrioz (zilurgisertib) in Fibrodysplasia ossificans progressiva: new drug review](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/monograph/review) · [Markdown](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/monograph/review.md) - [Atebrioz (zilurgisertib) in Fibrodysplasia ossificans progressiva: prevalence by geography](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/prevalence) · [Markdown](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/prevalence.md) - [Atebrioz (zilurgisertib) in Fibrodysplasia ossificans progressiva: interactive budget impact model](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/budget-impact-model) · [Markdown](https://www.evicenter.com/drugs/atebrioz--fibrodysplasia-ossificans-progressiva/budget-impact-model.md)