In the 21st century, an increasing trend in the incidence and mortality of IPF has occurred globally (9,12,53). As the knowledge of this disease has increased, it is obvious that this information should also be transmitted to primary health care physicians. Not only is it important that physicians can make an early recognition of the disease, but it is also beneficial for end-life decisions as well as for organizing adequate palliative and terminal care for the patients.
The unpredictable disease course of IPF was apparent in this study. Simple clinical factors as well as indexes appear to be inadequate in predicting the outcome of the individual patient. Biomarkers in blood and in BAL fluid have been investigated and promising results on predicting prognosis and treatment response have been reported (32,169). In the future, biomarkers may provide a way of accurately diagnosing IPF with less invasive procedures, as well as monitoring disease progression and predicting both outcome and treatment responses. Biomarkers have also been investigated in the prediction of AExs, were these to be discovered, they would represent a significant advance (169). In addition, if the mysteries surrounding the pathophysiology of AEx were to be solved, it could provide ways for preventing and managing these life-threatening, in many cases, even fatal events.
Recently published results of gene sequencing indicated that multiple genetic factors might be involved in the different forms of disease progression in IPF (86). Hopefully, future
research will reveal additional information of the pathophysiologic and genetic variants in IPF and their relation to disease progression as well as to treatment outcomes. As a huge amount of work has been done in developing novel pharmacological treatments for IPF, one may be optimistic that more individual and efficacious therapies will be available, thus making it possible to reduce or even eliminate the need for lung transplantation in the future (66).
7 Conclusions
1. Higher age and lower DLco % as well as a higher CPI value and a higher GAP stage were significantly related to a poorer prognosis in this study. The DLco change in 12 months with a cut-off value of 22 % had the best accuracy in predicting survival less than 2 years. In addition, GAP staging was more accurate than CPI in predicting survival less than 2 years. GAP staging was good in assessing a mild stage of disease since 79.5 % of GAP stage I patients survived for over 5 years. GAP III patients had high mortality, but only a minority of the patients surviving less than 2 years had been considered to be in GAP stage III at baseline. It seems that the optimal applicability of the GAP staging in the clinic is in the initial evaluation of patients with IPF. During follow-up, the changes in CPI may provide important information on disease progression.
2. Current smokers developed IPF earlier and died at a younger age than either non-smokers or ex-non-smokers. Females had longer survival times than males, but males had a higher risk of death only when ex-smokers and non-smokers were compared. The number of comorbidities did not have any effect on survival, but COPD and CVDs as a comorbidity and the use of insulin were related to a higher risk of death.
3. Mortality from IPF has been increasing in the KUH area between the years 2002 and 2012. The overall mortality was higher in males, but males had more commonly comorbidities as the underlying cause of death, i.e. the disease-specific mortality for IPF was higher in females. Patients with a rapid disease course had more often an AEx preceding death in comparison with patients with a slower course of the disease.
The numbers of PF deaths have increased in Finland if one compares the years 1998 and 2015.
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