IntroductionPatients with progressive idiopathic fibrotic interstitial lung disease (ILD), such as those with idiopathic pulmonary fibrosis (IPF), can have an aggressive disease course, with a median survival of only 3–5 years from diagnosis. The palliative care needs of these patients are often unmet. There are calls for new models of care, whereby the patient’s usual respiratory clinician remains central to the integration of palliative care principles and practices into their patient’s management, but the optimal model of service delivery has yet to be determined.MethodsWe developed a novel, collaborative, multidisciplinary team (MDT) meeting between our palliative care, psychology and ILD teams with the principal aim of integrating specialist care to ensure the needs of persons with ILD, and their caregivers were identified and met by referral to the appropriate service. The objective of this study was to assess the effectiveness of this novel MDT meeting on the assessment of a patient’s palliative care needs.ResultsSignificant increases in advance care planning discussions were observed, in conjunction with increased referrals to community courses and teams, following introduction of this novel MDT.ConclusionsOur results suggest that our collaborative MDT is an effective platform to address patients’ unmet palliative care needs. Further work is required to explore the effect of our model on achieving the preferred place of death and reductions in unplanned hospital admissions.

Purpose: Pirfenidone and nintedanib are two novel antifibrotic agents licensed for the treatment IPF. Prior to being approved for use in England for patients with FVC >50% and <80%, these were made available for all IPF patients under the Mild Patient Program (MPP) and Patient In Need Scheme (PIN). Prescribing of these medications is restricted to specialist centers. We sought to characterize the population of patients prescribed antifibrotics and determine the drug tolerability of these medications in the Northern hub of the Southwest of England regional ILD network.Methods: A retrospective analysis of all patients treated with antifibrotics between August 2012 and July 2017 was undertaken. Baseline characteristics including patient demographics and pulmonary physiology, in addition to drug tolerability and reasons for treatment cessation were collated. Data were compared using unpaired student’s t-test, Chi-squared, Mann–Whitney rank sum or ANOVA as appropriate. Logistic regression analysis evaluated clinical characteristics associated with discontinuation of pirfenidone therapy. P < 0.05 was considered statistically significant.Findings: A total of 164 patients, all with consensus diagnoses of IPF, were identified. Of these, 70.1% (115/164) received pirfenidone as their initial therapy. Baseline age, gender and pulmonary physiology did not differ significantly between groups. Drug discontinuation occurred most commonly due to adverse drug reactions events (ADRs) for both pirfenidone [40.0% (46/115)] and nintedanib [16.3% (8/49)]. Anorexia, rash and gastrointestinal disturbance were reported most commonly as the reason for cessation of pirfenidone; anorexia, nausea and weight loss for nintedanib. Duration of therapy prior to discontinuation because of ADRs did not differ significantly between medication groups but patients with a baseline FVC < 65% predicted, had a significantly shorter duration of pirfenidone prior to discontinuation due to ADRs, compared to those with a FVC 65–80% predicted. Multivariate logistic regression did not identify any independent baseline characteristics that predicted discontinuation of pirfenidone therapy prior to 52 weeks.Implications: Idiopathic pulmonary fibrosis (IPF) patients treated with nintedanib had comparable treatment emergent adverse event (TEAE) profiles in clinical practice to those reported in clinical trials. The TEAE profile of pirfenidone was higher than clinical trial data would suggest, although comparable to real-world datasets. Further work is required to explore the possible reasons underpinning this finding, including whether this is related to population co-morbidities or center threshold. No new safety concerns were identified.

Idiopathic pulmonary fibrosis is a progressive fibrosing interstitial lung disease for which there is no known cure. Currently available therapeutic options have been shown at best to slow the progression of the disease and thus there remains an urgent unmet need to identify new therapies. In this article, we will discuss the mechanisms of action, pre-clinical and clinical trial data surrounding inhibitors of the autotaxin-lysophosphatidic acid axis, which show promise as emerging novel therapies for fibrotic lung disease.

BackgroundLong-term nitrofurantoin (NF) treatment can result in pulmonary and hepatic injury. Current guidelines do not outline the type or frequency of monitoring required for detection of these injuries.AimsTo assess (1) awareness of NF complications among prescribers, (2) monitoring practice and (3) to describe the pulmonary sequelae of NF-related complications.Methods(1) Electronic questionnaire to prescribers, interrogating prescribing/monitoring practices and awareness of complications; (2) Case-note review (June-July 2020) of NF monitoring among general practitioners (GPs) in our local clinical commissioning group; (3) Case review of patients diagnosed with nitrofurantoin-induced interstitial lung disease (NFILD) at our interstitial lung disease (ILD) centre (2014-2020).Results125 prescribers of long-term NF responded to the questionnaire (82% GPs; 12% urologists). Many were unaware of the potential for liver (42%) and lung (28%) complications. 41% and 53% never monitored for these, respectively. Only 53% of urologists believed themselves responsible for arranging monitoring, whilst nearly all GPs believed this to be the prescriber’s responsibility (94%). One third of all respondents considered current British National Formulary (BNF) guidelines “not at all sufficient/clear”, with mean clarity scoring of 2.2/5. Amongst NFILD patients (n=46), NF had been prescribed most often (70%) for treatment of recurrent UTI and 59% (n=27) were prescribed for >6 months. Upon withdrawal of the medication 61% displayed resolution (completely/minimal fibrosis), whilst 16% of patients had progressive lung fibrosis.ConclusionNF can cause marked or irreversible lung complications and there is currently a shortfall in awareness and monitoring. Existing monitoring guidelines should be augmented.