The European Journal of Health Economics

, Volume 17, Supplement 1, pp 109–117 | Cite as

Social/economic costs and health-related quality of life in patients with scleroderma in Europe

  • Julio López-BastidaEmail author
  • Renata Linertová
  • Juan Oliva-Moreno
  • Pedro Serrano-Aguilar
  • Manuel Posada-de-la-Paz
  • Panos Kanavos
  • Domenica Taruscio
  • Arrigo Schieppati
  • Georgi Iskrov
  • Márta Péntek
  • Claudia Delgado
  • Johann Mathias von der Schulenburg
  • Ulf Persson
  • Karine Chevreul
  • Giovanni Fattore
  • The BURQOL-RD Research Network
Original Paper



The aim of this study was to determine the economic burden from a societal perspective and the health-related quality of life (HRQOL) of patients with systemic sclerosis (SSc; scleroderma) in Europe.


We conducted a cross-sectional study of patients with SSc (involving both localised and systemic sclerosis) from Germany, Italy, Spain, France, the UK, Hungary and Sweden. Data on demographic characteristics, healthcare resource utilisation, informal care, labour productivity losses and HRQOL were collected from the questionnaires completed by patients or their caregivers. HRQOL was measured with the EuroQol 5-domain (EQ-5D) questionnaire.


A total of 589 patients completed the questionnaire. The rate of patients with localised scleroderma, limited cutan and diffuse cutan SSc were 28, 68 and 4 %, respectively. Average annual costs varied from country to country and ranged from € 4607 to € 30,797 (reference year: 2012). Estimated direct healthcare costs ranged from € 1413 to € 17,300; direct non-healthcare costs ranged from € 1875 to € 4684 and labour productivity losses ranged from € 1701 to € 14,444. The mean EQ-5D index score for adult SSc patients varied from 0.49 to 0.75 and the mean EQ-5D visual analogue scale score was between 58.72 and 65.86.


The main strengths of this study lie in our bottom-up approach to costing and our evaluation of SSs patients from a broad societal perspective. This type of analysis is very unusual in the international literature on rare diseases in comparison with other illnesses. We concluded that SSc patients incur considerable societal costs and experience substantial deterioration in HRQOL.


Scleroderma Systemic sclerosis Localised scleroderma Cost of illness Health-related quality of life Economic burden 



The authors wish to thank: National Alliance of People with Rare Diseases (NAPRD), Bulgaria; Alliance Maladies Rares, France; ACHSE, Germany; Hungarian Federation of People with Rare and Congenital Diseases (RIROSZ), Hungary; Federazione Italiana Malattie Rare (UNIAMO), Italy; the Consulta Nazionale delle Malattie Rare, Italy; Rare Diseases Sweden; Federación Española de Efermedades Raras (FEDER), Spain; Rare Disease UK and Rare Diseases Europe (EURORDIS); FESCA (Federation of European Scleroderma Associations); Association des Sclérodermiques de France; Sklerodermie Selbsthilfe e.V., Germany; Országos Scleroderma Közhasznú Egyesület, Hungary; AILS—Associazione italiana lotta alla Sclerodermia and As.MA.RA—Associazione malattia rara Sclerodermia ed altre malattie rare “Elisabetta Giuffre”, Italy; Reumatikerförbundet, Sweden; Asociación Española de Esclerodermia and Asociación de Esclerodermia de Castellón, Spain; Raynaud’s and Scleroderma Association and Scleroderma Society, UK. Juan Oliva-Moreno and Julio López-Bastida are also grateful for the support of the project ECO2013-48,217-C2-2-R, “Impacto económico, sanitario y social de las enfermedades y los problemas de salud: información y herramientas para la evaluación de políticas públicas—Socioeconomic and healthcare impact of health problems: information and tools for the evaluation of public policies”.

Compliance with ethical standards


Supported by the Social/Economic Burden and Health-Related Quality of Life in Patients with Rare Diseases in Europe Project, which received funding from the European Union within the framework of the Health Programme [grant A101205]. The Executive Agency of the European Union is not responsible for any use that may be made of the information contained herein.

Conflict of interest

The authors declare that they have no conflicts of interest.

Supplementary material

10198_2016_789_MOESM1_ESM.docx (16 kb)
Supplementary material 1 (DOCX 16 kb)
10198_2016_789_MOESM2_ESM.doc (41 kb)
Supplementary material 2 (DOC 41 kb)


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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Julio López-Bastida
    • 1
    • 2
    Email author
  • Renata Linertová
    • 2
    • 3
  • Juan Oliva-Moreno
    • 2
    • 4
  • Pedro Serrano-Aguilar
    • 2
    • 5
  • Manuel Posada-de-la-Paz
    • 6
  • Panos Kanavos
    • 7
  • Domenica Taruscio
    • 8
  • Arrigo Schieppati
    • 9
  • Georgi Iskrov
    • 10
    • 11
  • Márta Péntek
    • 12
  • Claudia Delgado
    • 13
  • Johann Mathias von der Schulenburg
    • 14
  • Ulf Persson
    • 15
  • Karine Chevreul
    • 16
    • 17
    • 18
  • Giovanni Fattore
    • 19
  • The BURQOL-RD Research Network
  1. 1.Universidad de Castilla-La ManchaToledoSpain
  2. 2.Red de Investigación en Servicios Sanitarios en Enfermedades Crónicas (REDISSEC)MadridSpain
  3. 3.Fundación Canaria de Investigación Sanitaria (FUNCANIS)Las Palmas de Gran CanariaSpain
  4. 4.Universidad de Castilla-La ManchaToledoSpain
  5. 5.Evaluation and Planning Service at Canary Islands Health ServiceSanta Cruz de TenerifeSpain
  6. 6.Instituto de Investigación en Enfermedades Raras (IIER)SpainRDR and CIBERERMadridSpain
  7. 7.Health and Social Care Research CentreLondon School of Economics and Political ScienceLondonUK
  8. 8.National Center for Rare DiseasesIstituto Superiore di Sanità (ISS)RomeItaly
  9. 9.Centro di Ricerche Cliniche per Malattie Rare Aldo e Cele DaccòIstituto di Ricerche Farmacologiche Mario NegriRanica (Bergamo)Italy
  10. 10.Institute of Rare DiseasesPlovdivBulgaria
  11. 11.Department of Social Medicine and Public Health, Faculty of Public HealthMedical University of PlovdivPlovdivBulgaria
  12. 12.Department of Health EconomicsCorvinus University of BudapestBudapestHungary
  13. 13.Federación Española de Enfermedades Raras (FEDER)MadridSpain
  14. 14.Centre for Health Economics Research Hannover (CHERH)/Institute for Risk and InsuranceLeibniz Universität HannoverHannoverGermany
  15. 15.Swedish Institute for Health EconomicsLundSweden
  16. 16.URC Eco Ile de France, AP-HPParisFrance
  17. 17.Université Paris Diderot Sorbonne Paris Cité, ECEVE, UMRS 1123ParisFrance
  18. 18.INSERM, ECEVE, U1123ParisFrance
  19. 19.Centre for Research on Health and Social Care Management (CERGAS)Bocconi UniversityMilanItaly

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