Early Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis: A Narrative Review.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown aetiology. Patients typically present with symptoms of chronic dyspnoea and cough over a period of months to years. IPF has a poor prognosis, with an average life expectancy of 3-5 years from diagnosis if left untreated. Two anti-fibrotic medications (nintedanib and pirfenidone) have been approved for the treatment of IPF. These drugs slow disease progression by reducing decline in lung function. Early diagnosis is crucial to ensure timely treatment selection and improve outcomes. High-resolution computed tomography (HRCT) plays a major role in the diagnosis of IPF. In this narrative review, we discuss the importance of early diagnosis, awareness among primary care physicians, lung cancer screening programmes and early IPF detection, and barriers to accessing anti-fibrotic medications.

When should palliative care be introduced for people with progressive fibrotic interstitial lung disease? A meta-ethnography of the experiences of people with end-stage interstitial lung disease and their family carers.

BACKGROUND: Little is currently known about the perspectives of people with interstitial lung disease and their carers in relation to the timing of palliative care conversations. AIM: To establish patients' and carers' views on palliative care in interstitial lung disease and identify an optimum time to introduce the concept of palliative care. DESIGN: Meta-ethnography of qualitative evidence. The review protocol was prospectively registered with PROSPERO (CRD42021243179). DATA SOURCES: Five electronic healthcare databases were searched (Medline, Embase, CINAHL, Scopus and Web of Science) from 1st January 1996 to 31st March 2022. Studies were included that used qualitative methodology and included patients' or carers' perspectives on living with end-stage disease or palliative care. Quality was assessed using the Critical Appraisal Skills Programme checklist. RESULTS: About 1779 articles were identified by initial searches. Twelve met the inclusion criteria, providing evidence from 266 individuals across five countries. Three stages were identified in the illness journey of a person with interstitial lung disease: (1) Information seeking, (2) Grief and adjustment, (3) Fear of the future. Palliative care involvement was believed to be most appropriate in the latter two stages and should be prompted by changes in patients' health such as respiratory infections, onset of new symptoms, hospital admission, decline in physical function and initiation of oxygen. CONCLUSIONS: Patients and carers prefer referral to palliative care services to be prompted by changes in health status. Future research should focus on supporting timely recognition of changes in patients' health status and how to respond in a community setting.

Which factors influence the quality of end-of-life care in interstitial lung disease? A systematic review with narrative synthesis.

BACKGROUND: People dying from interstitial lung disease experience considerable symptoms and commonly die in an acute healthcare environment. However, there is limited understanding about the quality of their end-of-life care. AIM: To synthesise evidence about end-of-life care in interstitial lung disease and identify factors that influence quality of care. DESIGN: Systematic literature review and narrative synthesis. The review protocol was prospectively registered with PROSPERO (CRD42020203197). DATA SOURCES: Five electronic healthcare databases were searched (Medline, Embase, PubMed, Scopus and Web of Science) from January 1996 to February 2021. Studies were included if they focussed on the end-of-life care or death of patients with interstitial lung disease. Quality was assessed using the Critical Appraisal Skills Programme checklist for the relevant study design. RESULTS: A total of 4088 articles were identified by initial searches. Twenty-four met the inclusion criteria, providing evidence from 300,736 individuals across eight countries. Most patients with interstitial lung disease died in hospital, with some subjected to a high burden of investigations or life-prolonging treatments. Low levels of involvement with palliative care services and advance care planning contributed to the trend of patients dying in acute environments. This review identified a paucity of research that addressed symptom management in the last few days or weeks of life. CONCLUSIONS: There is inadequate knowledge regarding the most appropriate location for end-of-life care for people with interstitial lung disease. Early palliative care involvement can improve accordance with end-of-life care wishes. Future research should consider symptom management at the end-of-life and association with location of death.

Rheumatoid arthritis related interstitial lung disease - improving outcomes over 25 years: a large multicentre UK study.

OBJECTIVE: This study explores whether the prognosis of interstitial lung disease in rheumatoid arthritis (RA-ILD) has improved over time and assesses the potential influence of drug therapy in a large multicentre UK network. METHODS: We analysed data from 18 UK centres on patients meeting criteria for both RA and ILD diagnosed over a 25-year period. Data included age, disease duration, outcome and cause of death. We compared all cause and respiratory mortality between RA controls and RA-ILD patients, assessing the influence of specific drugs on mortality in four quartiles based on year of diagnosis. RESULTS: A total of 290 RA-ILD patients were identified. All cause (respiratory) mortality was increased at 30% (18%) compared with controls 21% (7%) (P =0.02). Overall, prognosis improved over quartiles with median age at death rising from 63 years to 78 years (P =0.01). No effect on mortality was detected as a result of DMARD use in RA-ILD. Relative risk (RR) of death from any cause was increased among patients who had received anti-TNF therapy [2.09 (1.1-4.0)] P =0.03, while RR was lower in those treated with rituximab [0.52(0.1-2.1)] or mycophenolate [0.65 (0.2-2.0)]. Patients receiving rituximab as their first biologic had longer three (92%), five (82%) and seven year (80%) survival than those whose first biologic was an anti-TNF agent (82%, 76% and 64%, respectively) (P =0.037). DISCUSSION: This large retrospective multicentre study demonstrates survival of patients with RA-ILD has improved. This may relate to the increasing use of specific immunosuppressive and biologic agents.

Optimising the management of patients with difficult asthma.

Asthma affects 5.4 million people in the UK, around 1 in 12 of the population. Between 5 and 10% of asthma (depending on the definition used) is categorised as difficult asthma, a term which generally refers to patients who continue to experience symptoms and frequent exacerbations despite the prescription of high-dose asthma therapy. Difficult asthma is an indication for specialist review by an appropriate respiratory physician, but close liaison between primary, secondary and tertiary care is critical and it is therefore important that primary care health professionals should be aware of the principles of management. One of the most important questions to ask is whether the individual with difficult asthma is taking their treatment Identifying this, however, is not easy. GPs could assess prescription uptake, looking for low use of preventers and excess use of short-acting bronchodilators. Newer means of assessing adherence have been developed. Inhaler devices that can monitor completion and timing of actuations have been produced. Meters that measure FeNO are available. A recent UK study found that 12 out of 100 patients referred for difficult asthma did not have reversible airflow obstruction or a history suggestive of asthma. Diagnoses included COPD, cystic fibrosis, cardiomyopathy, respiratory muscle dysfunction and severe anxiety with vocal cord dysfunction.