IPF is a rare disease, affecting only a small proportion of the population. A rare disease can be classified as such if it affects fewer than 200 000 people in the US1 or fewer than 1 in 2 000 people in Europe.2
There are no reliable, large-scale data sets on the incidence or prevalence of IPF. However, several small-scale studies3-7 (see table below) have suggested that the:
However, the true number of patients suffering from this disease is thought to be higher as IPF is underdiagnosed and often misdiagnosed.8
While these incidence and prevalence estimates give a useful guide, care must be taken in interpreting the data as different populations were used in each study, and estimates vary considerably depending on the definition applied.
The incidence of IPF is increasing, with incidence in the UK more than doubling between 1990 and 20033 and incidence in the USA predicted to double between 2005 and 2030.6 These figures take into account the expected increase in population sizes as well as the gradual ageing of the population, so the reasons for this apparent rise are not yet understood.
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