Idiopathic Pulmonary Fibrosis
Lung transplantation is the only option known to increase survival time in those with IPF, but is only performed in a minority of cases (Caminati 2017). Other treatment approaches are palliative and aim to alleviate symptoms, improve quality of life, manage acute exacerbations, and possibly slow disease progression. The best supportive care for an individual with IPF may include not only medication and disease monitoring but also physical therapies, occupational therapy, and counseling (Ferrara 2018; Scelfo 2017).
Shortness of breath and cough are the main symptoms of IPF and contribute substantially to declining quality of life (Scelfo 2017; Ferrara 2018).
Comprehensive pulmonary rehabilitation, with exercise and educational components, is helpful in managing shortness of breath, increasing ability to exercise, and improving quality of life (Ferrara 2018; Shaw 2017; Caminati 2017). Oxygen administration during exertion may also be a consideration for those who only experience breathing difficulties with physical activity, but its optimal timing and dosage are still unclear (Ferrara 2018). Furthermore, oxygen therapy can interfere with mobility and psychosocial interactions, and may have an overall negative impact on quality of life (Shaw 2017).
Typical cough-suppressing medications are generally not effective for IPF-associated cough, but oral corticosteroids such as prednisone may be beneficial in some cases (Ferrara 2018; Shaw 2017). Gabapentin (Neurontin), an anti-seizure medication, has been shown to be beneficial in cases of neurologically induced cough and has been proposed as a possible option for treating IPF-related cough (Atreya 2016; Ferrara 2018; Goa 1993). Sedatives like opioid and benzodiazepine drugs may be helpful as palliative therapies in alleviating both cough and shortness of breath in the end stages of IPF (Ferrara 2018; Shaw 2017).
Weight loss may be due to decreased appetite, increased energy demands of labored breathing, or gastrointestinal side effects of medications. The possibility of extra nutritional demands and the use of supplemental nutrition should be explored in cases involving substantial weight loss (Ferrara 2018).
Patients with IPF frequently suffer from anxiety and depression, which can contribute independently to a reduced quality of life. Anxiety and depression are closely correlated with breathlessness and disease severity, and the progressive nature of the disease can affect emotional well-being of the patients; treatment of anxiety and depression have to be addressed as soon as problems appear. In addition, antidepressant medications may be helpful in some cases (Ferrara 2018; Shaw 2017). Perhaps most importantly, psychological counseling should be offered soon after diagnosis (Shaw 2017; Lindell 2017). The importance of supporting patients' emotional well-being is often overlooked. IPF patients and their caregivers need guidance in understanding the condition's prognosis (Scelfo 2017; Lindell 2017; Caminati 2017).
Vaccination against influenza and pneumonia is important for IPF patients as well (National Heart, Lung, and Blood Institute 2018).
Effective treatments for IPF have historically been elusive. Despite the role of inflammation in triggering fibrotic activity, anti-inflammatory approaches have been consistently ineffective (Sgalla 2018). Two anti-fibrotic agents—pirfenidone (Esbriet) and nintedanib (Ofev)—were approved in 2014 based on evidence of benefits in treating IPF (Sharif 2017; Scelfo 2017; Bando 2016; McCormack 2015). Both drugs may slow disease progression, but do not completely stop or reverse the underlying disease processes.
- Pirfenidone. Although the exact mechanism of action is still uncertain, pirfenidone inhibits fibroblast and collagen synthesis. It also has anti-inflammatory and oxidative stress-reducing activities that may contribute to its benefits (Sgalla 2018). Findings from several randomized placebo-controlled trials suggest pirfenidone may slow disease progression (Azuma 2005; Taniguchi 2010; Noble 2011). In an uncontrolled trial, subjects from a previous trial who had received placebo were subsequently treated with pirfenidone and reported positive findings (Costabel 2014).
- Nintedanib. Nintedanib inhibits enzymes in the tyrosine kinase family that are involved in the formation of fibrotic tissue in the lungs (Sgalla 2018). In a clinical study in which 62 IPF patients used nintedanib in a compassionate use program, the drug stabilized IPF progression in 63% of patients after six months (Bonella 2016).
The most frequent and important adverse side effects of these medications are gastrointestinal in nature, including indigestion, nausea, and diarrhea; in addition, pirfenidone use can cause light sensitivity (Jiang 2012; Margaritopoulos 2016; Hughes 2016). The available data so far does not favor one over the other in terms of efficacy or safety, but does suggest that treatment with either agent may be more helpful if started early (Scelfo 2017). The potential advantage of using pirfenidone and nintedanib together is currently under investigation (Sgalla 2018).
A lung transplant may increase survival time and is an important option in appropriate patients with moderate-to-advanced IPF (Sharif 2017; Kistler 2014). Nonetheless, post-transplant survival in IPF is lower than in other pulmonary diseases, at approximately 75% at one year, 59% at two years, 47% at five years, and 24% at 10 years (Kistler 2014). In addition, despite being the most common condition represented on waiting lists for lung transplant, fewer than 20% of patients with IPF receive a lung transplant, reflecting the long waiting lists and high rate of death among IPF patients on them (Caminati 2017; Lindell 2017).
Treatment of Related Conditions
IPF patients frequently suffer from other chronic conditions. Emphysema, lung cancer, pulmonary hypertension, sleep apnea, gastroesophageal reflux, and coronary artery disease are among the most common co-occurring conditions (Scelfo 2017). Medical management of these co-occurring conditions may have a positive impact on the course of IPF. For example, in IPF patients with sleep apnea, treatment with continuous positive airway pressure (CPAP) appears to improve sleep and quality of life (Mermigkis 2015). Observational evidence suggests proton pump inhibitor treatment for those with gastroesophageal reflux may slow IPF progression and increase survival time, and some research findings suggest they may even be helpful in IPF patients without evidence of gastroesophageal reflux (Ghebre 2016). The use of bronchodilators may be helpful in those with combined IPF and emphysema, but their impact on symptoms and prognosis have not yet been established (Sgalla 2018; Zhang 2016).
Pulmonary hypertension is a frequent finding and contributor to symptoms and mortality in patients with IPF. It may occur as a result of fibrosis in pulmonary vessels or blood vessel constriction in response to low oxygen levels. Sildenafil (Viagra) is a blood vessel dilator with an effect on pulmonary vessels. Findings from some, but not all, studies suggest sildenafil has a positive impact on breathlessness, ability to exercise, and quality of life in IPF patients with pulmonary hypertension (Han 2013; Zisman 2010; Jackson 2010; Collard 2007).
Management of Acute Exacerbation
Sudden worsening of symptoms, or acute exacerbation, is a significant problem in IPF. It can be difficult to distinguish from infectious pneumonia and other respiratory problems and is a frequent cause of fatal respiratory failure. An accurate assessment will guide therapy. Management of acute exacerbation typically involves mechanical ventilation, intravenous fluids, high-dose steroids, morphine, and antibiotics as appropriate (Scelfo 2017).