BackgroundPeople with pulmonary fibrosis often experience a protracted time to diagnosis, high symptom burden and limited disease information. This review aimed to identify the supportive care needs reported by people with pulmonary fibrosis and their caregivers.MethodsA systematic review was conducted according to PRISMA guidelines. Studies that investigated the supportive care needs of people with pulmonary fibrosis or their caregivers were included. Supportive care needs were extracted and mapped to eight pre-specified domains using a framework synthesis method.ResultsA total of 35 studies were included. The most frequently reported needs were in the domain of information/education, including information on supplemental oxygen, disease progression and prognosis, pharmacological treatments and end-of-life planning. Psychosocial/emotional needs were also frequently reported, including management of anxiety, anger, sadness and fear. An additional domain of “access to care” was identified that had not been specified a priori; this included access to peer support, psychological support, specialist centres and support for families of people with pulmonary fibrosis.ConclusionPeople with pulmonary fibrosis report many unmet needs for supportive care, particularly related to insufficient information and lack of psychosocial support. These data can inform the development of comprehensive care models for people with pulmonary fibrosis and their loved ones.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing lung disease that leads to unrelenting dyspnoea and chronic cough, and ultimately respiratory failure [1]. IPF is characterised by a variable disease course that remains difficult to predict for an individual at diagnosis [2]. In the current era, with the advent of anti-fibrotic therapy which can slow disease progression, it is increasingly important to identify patients with early disease and to target those patients who are at most risk of rapid decline [3]. However, despite multiple studies proposing novel potential prognostic biomarkers, the current American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Society guideline statements dismissed the use of these biomarkers except in a research capacity [3,4].Recently, SCOTT et al. [5] have suggested that peripheral blood monocyte count may be a powerful biomarker capable of predicting poorer prognosis amongst IPF patients. In a retrospective analysis using transcriptome microarray data, the investigators found that estimated CD14+ monocyte percentages above the mean were associated with shorter transplant-free survival times. Using a threshold monocyte value of 0.95×10 3 per µL or greater, the investigators were able to validate their exploratory analysis using several independent cohorts. Higher absolute CD14+ monocyte counts were found in the COMET cohort in patients who had progressive disease and in those that were classified as high-risk in the Yale cohort. Higher absolute monocyte counts were found to be associated with increased risk of mortality in several other larger validation cohorts. This finding remained significant following adjustment for forced vital capacity (FVC) and the gender, age and physiology index in each of the COMET, Stanford and Northwestern cohorts.We sought to reproduce this finding using data from the Australian IPF Registry (AIPFR). The national AIPFR is investigator-led, prospectively acquired and was established in 2012 with the goal of facilitating research into IPF as well as to provide further insight into the epidemiology and management of IPF in Australia [6]. Baseline routine full blood count tests with cell differentials performed on patients recruited into the registry were extracted for analysis. A total of 231 patients from three Australian states (New South Wales, Victoria and South Australia) were included.Clinical data had been prospectively entered into the Registry with a follow up duration of 2.4 (1.3-3.3) years. The majority of patients were male (71%) with a mean±SD age of 69.9±8.3 years. Patients were found initially to have mild to moderate disease with a mean FVC of 80.3±22.0% predicted and a mean diffusing capacity of the lung for carbon monoxide of 48.2±16.8% predicted. Mean blood monocyte counts were significantly higher in the 75 patients who died compared to the 156 patients who remained alive after the follow-up period, although the difference was clinically small (0.66 (0.5-0.9...