Using forced vital capacity (FVC) in the clinic to monitor patients with idiopathic pulmonary fibrosis (IPF): pros and cons.

INTRODUCTION: Forced vital capacity (FVC) decline is predictive of mortality in patients with idiopathic pulmonary fibrosis (IPF) and has been used as a clinical trial endpoint to define disease progression. How to interpret FVC findings in an individual patient with IPF in the real-world setting amid uncertainty about the measurement accuracy and variability has not been well established. AREAS COVERED: This review highlights the challenges and limitations of using FVC in the clinic to monitor disease progression in patients with IPF. Spirometry is noninvasive, relatively simple, and inexpensive. FVC measurements provide evidence for trends over time in patients with IPF. When using FVC in the clinic, several important challenges and limitations, including visit-to-visit variability, dependence on patient effort, inconsistent quality control, limitations on accuracy, and the influence of comorbidities and pretest factors, must be considered. Recent studies suggest the potential for home spirometry devices to facilitate more frequent collection of data and perhaps demonstrate more accurate trends. EXPERT OPINION: Measuring FVC decline in the clinic has an important role in monitoring disease progression in patients with IPF, but additional measures of disease progression should be considered along with FVC to facilitate decision-making about disease management.

Development and validation of the Chronic Obstructive Pulmonary Disease Assessment Questionnaire (COPD-AQ).

AIMS: To develop a practical patient-completed chronic obstructive pulmonary disease assessment questionnaire (COPD-AQ) to improve COPD assessment and management in primary care, based on the concept of COPD stability. METHODS: An Expert Working Group defined parameters of COPD stability and a 10-item Physician's Global Assessment was established. A 21-item COPD-AQ was developed and validated in a cross-sectional, non-randomised study of patients with COPD (n=395). Items most discriminative of stability status (stable/unstable) were selected to produce a 5-item COPD-AQ, which was then validated. RESULTS: In the development sample, internal consistency reliability of the 5-item COPD-AQ was 0.74 (n=296). The COPD-AQ discriminated between stability groups based on physician assessment (F=44.26; p<0.0001) and post-bronchodilator spirometry measures (F=2.92; p<0.05). A questionnaire score >20 (range: 5.0-25.0) had a specificity of 82.9% and sensitivity of 64.7%. CONCLUSIONS: The 5-item COPD-AQ proved a practical tool for assessing COPD status and was sufficiently simple for routine clinical use. However, overall validation was limited by small numbers of patients in the validation sample. Difficulties also existed over using the term 'stability' to define COPD status. COPD-AQ was not progressed further, but this work will prove valuable in the future development of a global questionnaire to improve COPD management in primary care.

Pirfenidone safety and adverse event management in idiopathic pulmonary fibrosis.

Pirfenidone is one of two approved therapies for the treatment of idiopathic pulmonary fibrosis (IPF). Randomised controlled clinical trials and subsequent post hoc analyses have demonstrated that pirfenidone reduces lung function decline, decreases mortality and improves progression-free survival. Long-term extension trials, registries and real-world studies have also shown similar treatment effects with pirfenidone. However, for patients with IPF to obtain the maximum benefits of pirfenidone treatment, the potential adverse events (AEs) associated with pirfenidone need to be managed. This review highlights the well-known and established safety profile of pirfenidone based on randomised controlled clinical trials and real-world data. Key strategies for preventing and managing the most common pirfenidone-related AEs are described, with the goal of maximising adherence to pirfenidone with minimal AEs.

Interstitial lung disease: raising the index of suspicion in primary care.

Interstitial lung disease (ILD) describes a group of diseases that cause progressive scarring of the lung tissue through inflammation and fibrosis. The most common form of ILD is idiopathic pulmonary fibrosis, which has a poor prognosis. ILD is rare and mainly a disease of the middle-aged and elderly. The symptoms of ILD-chronic dyspnoea and cough-are easily confused with the symptoms of more common diseases, particularly chronic obstructive pulmonary disease and heart failure. ILD is infrequently seen in primary care and a precise diagnosis of these disorders can be challenging for physicians who rarely encounter them. Confirming a diagnosis of ILD requires specialist expertise and review of a high-resolution computed tomography scan (HRCT). Primary care physicians (PCPs) play a key role in facilitating the diagnosis of ILD by referring patients with concerning symptoms to a pulmonologist and, in some cases, by ordering HRCTs. In our article, we highlight the importance of prompt diagnosis of ILD and describe the circumstances in which a PCP's suspicion for ILD should be raised in a patient presenting with chronic dyspnoea on exertion, once more common causes of dyspnoea have been investigated and excluded.