Estimating the burden of scleroderma disease in Spain.
OBJECTIVE
Scleroderma (systemic sclerosis) is a rare disease that results in great suffering. We estimated the burden of disease posed by scleroderma and its relative importance in the health of the Spanish population.


METHODS
We estimated scleroderma-based burden of disease following procedures developed for the Global Burden of Disease study to ensure comparability.


RESULTS
Despite its low prevalence, scleroderma generated 1732 disability-adjusted life-years (DALY) in Spain in 2001, comprising 562 (32%) years of life lost and 1170 (68%) years lived with disability. Most scleroderma-related DALY (73%) occurred in the population aged 15-54 years. Estimated DALY accounted for 0.59% of other musculoskeletal disorder-related DALY in the European A subregion (countries with low mortality rate in both adults and children in the World Health Organization classification), a significant value in the overall burden of disease.


CONCLUSIONS
The burden of scleroderma in Spain was high, with disability being the major contributing factor. Burden of disease is an important measure in rare diseases, and may be an important indicator to be considered as a health unit in developed countries.
Villaverde-Hueso
Ana
A
Instituto de Investigacion de Enfermedades Raras, Instituto de Salud Carlos III, Sinesio Delgado 6-8, Madrid, Spain.
Sánchez-Valle
Elena
E
Alvarez
Elena
E
Morant
Consuelo
C
Carreira
Patricia E
PE
Martín-Arribas
María Concepción
MC
Gènova
Ricard
R
Ramírez-González
Alejandro
A
de la Paz
Manuel Posada
MP
D000293:Adolescent; D000328:Adult; D000368:Aged; D017281:Cost of Illness; D004185:Disability Evaluation; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019057:Quality-Adjusted Life Years; D012595:Scleroderma, Systemic / Q000191:economics / Q000453:epidemiology* / Q000503:physiopathology; D013030:Spain / Q000453:epidemiology