It is clear that pharmacological treatments aimed at reversing the progressive fibrosis that characterizes IPF have been unsuccessful and often harmful because of significant adverse effects. Traditionally steroids have been used to treat IPF, although no clinical trial data to support their use exist. In the British Thoracic Society ILD Guidelines 2008, a recommendation to use triple therapy with prednisolone, azathioprine, and n-acetylcysteine was made [1]. However, subsequent international guidelines have been unable to recommend the routine use of immunosuppressive drugs and indeed concluded that there is no proven pharmacological therapy for IPF [8]. A recent randomized controlled trial of triple therapy (PANTHER-IPF) was stopped prematurely as patients treated with triple therapy had increased mortality, more serious adverse events, and drug discontinuations, without evidence of benefit. Many other disease-modifying therapies have been tried unsuccessfully and whilst there may be some benefit from the anti-fibrotic drug pirfenidone in selected patients, there is unfortunately no effective drug treatment for progressive IPF. Patients with IPF should be offered participation in well-designed clinical trials with the potential benefits it may bring to both them and other patients in terms of outcomes, symptoms, and quality of life made explicit, although countered by the potential for adverse effects and harm [8].

The use of long-term oxygen therapy (LTOT) has been recommended for patients with IPF who have resting hypoxemia using the same criteria as recommended for patients with COPD [1,8]. It remains unclear however in the absence of clinical trial data that the survival benefits of LTOT seen in patients with COPD can be extrapolated to patients with IPF.

Lung transplantation is an effective treatment for a minority of patients with IPF. In selected patients, transplantation can dramatically improve the disease trajectory, although timing of referral for assessment remains critical to prevent patients becoming too unwell for consideration of lung transplant or dying on the waiting list [13]. Unfortunately many patients with IPF are elderly and have comorbidities which make them unsuitable for transplantation.

The focus on symptom-centered management links with the concept of “best supportive care” as outlined in the British Thoracic Society guidelines and is clearly integral to effective palliative care for a disease which has no proven cure or ­disease-modifying treatment [1,8,12].

An important consideration in being able to proactively identify patients with progressive disease and worsening symptoms is careful monitoring using appropriate clinical evaluation, symptom scores, and quality of life assessments. This approach allows the clinical team to consider therapy choices but also helps patients understand their disease course. The standard objective measures of disease severity and progression are lung function as measured by forced vital capacity (FVC), gas transfer (TLco), oxygenation saturation, and 6 min walk distance. It is widely accepted that a fall in FVC of ≥10 % or TLco of ≥15 % over a 3–6 month period is indicative of disease progression and a poor outcome. It is clearly important however to ensure that the patient’s symptoms are assessed and quantified accurately. Breathlessness is almost universal in patients with IPF, although there is a paucity of data on the clinical utility of assessment tools, most of which were developed for assessment of COPD patients, in this group of patients. Breathlessness has been demonstrated to have a strong correlation with both quality of life and mortality in IPF [14]. Despite its prevalence and prominence, breathlessness has not been the focus of many studies of the natural history or treatment interventions in IPF. The use of a patient-reported measure of breathlessness, Dyspnea-12, has shown promise as a simple, reliable, and valid instrument in patients with ILD [15].

Whilst breathlessness in IPF is principally due to the physiological impact of fibrosis, reduced gas exchange, and ventilation-perfusion mismatching, other factors also influence the patient’s perception of breathlessness. In a cross-sectional study of patients with ILD some of whom had IPF, both depression and functional status, as measured by walk distance, were strongly associated with breathlessness [16]. The authors of this study speculated that treatment of depression and programmes of exercise may improve both breathlessness and quality of life.

A dry irritating cough can be a distressing symptom for IPF patients [17]. Cough in IPF is often refractory to anti-tussive therapies and it may be a predictor of progressive disease and mortality [18]. The mechanism of cough in IPF is poorly understood, although it may be due to heightened cough reflex sensitivity [19]. Other co-existent causes of cough, such as gastro-esophageal reflux, upper airway cough syndrome, asthma, and COPD should be considered. There is no IPF-specific tool to assess the severity or impact of a patient’s cough, although there appears to be a strong correlation between cough frequency and cough-related quality of life measures [20].

Fatigue in IPF can be a dominant symptom for some patients and can be exacerbated further by muscle deconditioning [17]. Many patients with IPF suffer from anxiety and depression, which can be compounded by the effects of chronic and debilitating symptoms, adverse effects of drug therapies, and fear of disease ­progression and death [16].

Health-related quality of life (HRQOL) in IPF is now recognized as an important measure to identify changes in the disease process and to quantify the impact of symptoms in individual patients. It is also being increasingly used as an outcome measure in clinical trials. Swigris et al., having recognized the lack of validated HRQOL tools in this disease, interviewed patients in order to identify themes and develop an IPF-specific tool ATAQ-IPF [17,21]. A modification (SQRQ-I) to the Saint George’s Respiratory Questionnaire has been proposed to be reliable for measuring health-related quality of life in patients with IPF [22]. More recently, the King’s brief interstitial lung disease questionnaire (K-BILD) has been proposed as a simple tool to assess HRQL in three domains of psychological impact, breathlessness/activities, and chest symptoms [23]. In a pilot study, HRQOL was found to be significantly lower in patients with IPF compared to other ILDs and was also lower in patients with IPF who were prescribed long-term oxygen therapy or immunosuppressant medication. Clearly more studies are needed to assess the longitudinal changes in HRQL using IPF-specific tools and the potential impact of interventions both in research trials and in clinical practice.

There are a number of approaches that form the foundation of “best supportive care” for patients with IPF. These include smoking cessation, addressing nutrition, treating right heart failure and influenza and pneumococcal vaccination that are likely to improve symptoms and prevent the development of complications. In addition, there are four principal symptoms that should be sought and considered in all patients with IPF.