IPF:

Idiopathic pulmonary fibrosis (IPF), the prototype of interstitial lung diseases, has the worst prognosis and is the only interstitial lung disease for which approved pharmacological treatments are available. Despite being considered a rare disease, IPF patients pose major challenges to both physicians and healthcare systems. It is estimated that a large number of IPF patients reside in BRIC countries (Brazil, Russia, India, and China) given their overall total population of approximately 3 billion inhabitants. Nevertheless, the limited availability of chest imaging in BRIC countries is considered a chief obstacle to diagnosis, since high-resolution computed tomography of the chest is the key diagnostic test for IPF. Further, obtaining reliable lung function tests and providing treatment access is difficult in the more rural areas of these countries. However, IPF might represent an opportunity for BRIC countries: the exponentially increasing demand for the enrollment of IPF patients in clinical trials of new drugs is predicted to face a shortage of patients – BRIC countries may thus play a crucial role in advancing towards a cure for IPF.

Keywords: BRIC countries; High-resolution computed tomography; Idiopathic pulmonary fibrosis; Nintedanib; Pirfenidone

COPD:

Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality across the globe. According to World Health Organization estimates, 65 million people have moderate to severe COPD. More than 3 million people died of COPD in 2005 corresponding to 5% of all deaths globally and it is estimated to be the third leading cause of death by 2030.[1] Most of the information available on COPD prevalence, morbidity and mortality comes from high-income countries. Even in those countries, accurate epidemiologic data on COPD are difficult and expensive to collect. However, it is known that low- and middle-income countries already shoulder much of the burden of COPD with almost 90% of COPD deaths taking place in these countries.[2,3] In this issue of Lung India, the joint ICS/NCCP (I) consensus guidelines for the diagnosis and management of COPD have been published[4] to facilitate the Indian practitioner in burden reduction, diagnosis and management of COPD.
Idiopathic pulmonary fibrosis (IPF) is a disease of unknown aetiology associated with progressive parenchymal fibrosis 1. Patients with IPF face substantial morbidity and mortality, and report substantially impaired quality of life 2. Lung transplant (LT) represents the lone intervention that potentially improves survival in IPF 3.

Pulmonary hypertension (PH) is evolving as an important factor that can adversely affect outcomes in chronic lung disease. In advanced chronic obstructive pulmonary disease (COPD), therapy directed at controlling the pulmonary artery pressure has been recommended 4. In fibrotic lung diseases more akin to IPF, such as sarcoidosis, secondary PH is common and is a marker for early death 5, 6.

Less is known about PH in IPF. Epidemiologically, several retrospective analyses indicate that PH in IPF may be frequent 7–9. Illustrating the emerging interest in PH and IPF, Ghofrani et al. 10 examined the impact of sildenafil on pulmonary haemodynamics in lung fibrosis and concluded that it caused pulmonary vasodilation and improved gas exchange. Others have also explored inhaled agents in IPF related to PH 11.

With the advent of newer options for treating PH, coupled with the lack of effective therapies for IPF, targeting PH appears attractive. However, before studying interventions it is important to define the prevalence and extent of this process. With improved information regarding the prevalence of and clinical factors associated with PH in IPF, clinicians can better determine whom to evaluate for PH, and researchers can design more appropriate clinical trials. Thus, to explore the frequency of PH in IPF, the present authors retrospectively analysed the United States LT registry. The specific objectives were to describe the prevalence of PH in IPF, to assess the severity of PH in this population and to identify clinical variables that correlated with PH