Mortality and survival in idiopathic pulmonary fibrosis: a systematic review and meta-analysis.

Background: There are substantial advances in diagnosis and treatment for idiopathic pulmonary fibrosis (IPF), but without much evidence available on recent mortality and survival trends. Methods: A narrative synthesis approach was used to investigate the mortality trends, then meta-analyses for survival trends were carried out based on various time periods. Results: Six studies reported the mortality data for IPF in 22 countries, and 62 studies (covering 63 307 patients from 20 countries) reported survival data for IPF. Age-standardised mortality for IPF varied from ∼0.5 to ∼12 per 100 000 population per year after year 2000. There were increased mortality trends for IPF in Australia, Brazil, Belgium, Canada, Czech Republic, Finland, France, Germany, Hungary, Italy, Lithuania, the Netherlands, Poland, Portugal, Spain, Sweden and UK, while Austria, Croatia, Denmark, Romania and the USA showed decreased mortality trends. The overall 3-year and 5-year cumulative survival rates (CSRs) were 61.8% (95% CI 58.7-64.9; I2=97.1%) and 45.6% (95% CI 41.5-49.7; I2=97.7%), respectively. Prior to 2010, the pooled 3-year CSR was 59.9% (95% CI 55.8-64.1; I2=95.8%), then not significantly (p=0.067) increased to 66.2% (95% CI 62.9-69.5; I2=92.6%) in the 2010s decade. After excluding three studies in which no patients received antifibrotics after year 2010, the pooled 3-year CSRs significantly (p=0.039) increased to 67.4% (95% CI 63.9-70.9; I2=93.1%) in the 2010s decade. Discussion: IPF is a diagnosis associated with high mortality. There was no observed increasing survival trend for patients with IPF before year 2010, with then a switch to an improvement, which is probably multifactorial.

Essential Features of an Interstitial Lung Disease Multidisciplinary Meeting: An International Delphi Survey.

Rationale: The interstitial lung disease (ILD) multidisciplinary meetings (MDM), composed of pulmonologists, radiologists, and pathologists, is integral to the rendering of an accurate ILD diagnosis. However, there is significant heterogeneity in the conduct of ILD MDMs, and questions regarding their best practices remain unanswered. Objectives: To achieve consensus among ILD experts on essential components of an ILD MDM. Methods: Using a Delphi methodology, semi-structured interviews with ILD experts were used to identify key themes and features of ILD MDMs. These items informed two subsequent rounds of online questionnaires that were used to achieve consensus among a broader, international panel of ILD experts. Experts were asked to rate their level of agreement on a five-point Likert scale. An a priori threshold for consensus was set at a median score 4 or 5 with an interquartile range of 0. Results: We interviewed 15 ILD experts, and 102 ILD experts participated in the online questionnaires. Five items and two exploratory statements achieved consensus on being essential for an ILD MDM following two questionnaire rounds. There was consensus that the presence of at least one radiologist, a quiet setting with a visual projection system, a high-quality chest high-resolution computed tomography, and a standardized template summarizing collated patient data are essential components of an ILD MDM. Experts also agreed that it would be useful for ILD MDMs to undergo an annual benchmarking process and a validation process by fulfilling a minimum number of cases annually. Twenty-seven additional features were considered to be either highly desirable or desirable features based on the degree of consensus. Although our findings on desirable features are similar to the current literature, several of these remain controversial and warrant further research. The study also showed an agreement among participants on several future concepts to improve the ILD MDM, such as performing regular self-assessments and conducting research into shared practices to develop an international expert guideline statement on ILD MDMs. Conclusions: This Delphi study showed consensus among international ILD experts on essential and desirable features of an ILD MDM. Our data represent an important step toward potential collaborative research into future standardization of ILD MDMs.

Barriers and facilitators to best care for idiopathic pulmonary fibrosis in Australia.

BACKGROUND AND OBJECTIVE: In Australia, little is known about delivery of care for people with idiopathic pulmonary fibrosis (IPF). This study examined the organization of IPF care across Australia, how it aligns with guidance for best practice, and identified barriers and facilitators to best care. METHODS: Data on the organization of IPF care in Australia were collected from public hospitals using a study-specific questionnaire between February and July 2020. Semi-structured telephone interviews were conducted with respiratory physicians from around Australia between April and December 2020. Interviews were transcribed verbatim and thematic analysis was undertaken. RESULTS: Almost all hospitals (n = 38, 97%) held multidisciplinary meetings (MDMs) for diagnosing IPF, with 90% of multidisciplinary teams including expert respiratory physicians and radiologists; however, rheumatologists, interstitial lung disease nurses and a histopathologist were often not available. More than 90% of institutions had access to oxygen therapy, pulmonary rehabilitation and advanced care planning, but access to psychological support and clinical trials was limited (53% and 58%, respectively). Fifteen respiratory physicians (27% regional) were interviewed. Approaches to diagnosis, treatment and access to referral services were generally consistent with best practice guidance; however, regional respondents reported barriers related to inadequate staffing, lack of a nurse coordinator, inadequate access to clinical trials and funding models. Telehealth technologies were perceived as facilitators to best care. CONCLUSION: Clinical management of IPF in Australia generally aligns with best practice guidance, but there may be some inequity of access to specialist services, particularly in regional areas, that should be addressed to ensure optimal care for all.

Diagnosis of myositis-associated interstitial lung disease: Utility of the myositis autoantibody line immunoassay.

OBJECTIVES: The detection of myositis autoantibodies (MA) in patients with interstitial lung disease (ILD) has major implications for diagnosis and management, especially amyopathic and forme frustes of idiopathic inflammatory myositis-associated ILD (IIM-ILD). Use of the MA line immunoblot assay (MA-LIA) in non-rheumatological cohorts remains unvalidated. We assessed the diagnostic performance of the MA-LIA and explored combined models with clinical variables to improve identification of patients with IIM-ILD. METHODS: Consecutive patients referred to a specialist ILD clinic, with ILD-diagnosis confirmed at multidisciplinary meeting, and MA-LIA performed within six months of baseline were included. Pre-specified MA-LIA thresholds were evaluated for IIM-ILD diagnosis. RESULTS: A total 247 ILD patients were included (IIM-ILD n = 12, non-IIM connective tissue disease-associated ILD [CTD-ILD] n = 52, idiopathic interstitial pneumonia [IIP] n = 115, other-ILD n = 68). Mean age was 64.8 years, with 45.3% female, mean FVC 75.5% and DLCO 59.2% predicted. MA were present in 13.8% overall and 83.3% of IIM-ILD patients. The most common MA in IIM-ILD and non-IIM ILD patients were anti-Jo-1 (prevalence 40%) and anti-PMScl (29.2%) autoantibodies respectively. The pre-specified low-positive threshold (>10 signal intensity) had the highest discriminative capacity for IIM-ILD (AUC 0.86). Combining MA-LIA with age, gender, clinical CTD-manifestations and an overlap non-specific interstitial pneumonia/organising pneumonia pattern on HRCT improved discrimination for IIM-ILD (AUC 0.96). CONCLUSION: The MA-LIA is useful to support a diagnosis of IIM-ILD as a complement to multi-disciplinary ILD assessment. Clinical interpretation is optimised by consideration of the strength of the MA-LIA result together with clinical and radiological features of IIM-ILD.

Contemporary Concise Review 2020: Interstitial lung disease.

The year 2020 was one like no other, as we witnessed the far-reaching impact of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) global pandemic. Yet despite an unprecedented and challenging year, global research in interstitial lung disease (ILD) continued to break new grounds. Research progress has led to an improved understanding in new diagnostic tools and potential biomarkers for ILD. Studies on the role of antifibrotic therapies, newer therapeutic agents, supportive care strategies and the impact of coronavirus disease 2019 (COVID-19) continue to reshape the management landscape of ILD. In this concise review, we aim to summarize the key studies published in 2020, highlighting their impact on the various aspects of ILD.

Baseline Characteristics and Survival of an Australian Interstitial Pneumonia with Autoimmune Features Cohort.

BACKGROUND AND OBJECTIVE: The research term "interstitial pneumonia with autoimmune features" (IPAF) encompasses interstitial lung disease (ILD) patients with autoimmune features not meeting diagnostic criteria for a defined connective tissue disease (CTD). It remains unclear if IPAF is a distinct disease entity with implications for management and prognosis. We describe an Australian IPAF population and compare their baseline characteristics and outcomes with distinct cohorts of idiopathic interstitial pneumonia (IIP), CTD-ILD, and unclassifiable ILD. METHODS: Review of 291 consecutive patients attending a specialist ILD clinic was performed. Patients with a diagnosis of IIP, CTD-ILD, and unclassifiable ILD by ILD-multidisciplinary meeting (ILD-MDM) were included. Patients meeting the IPAF criteria were identified. Baseline clinical data, survival, and progression were compared between ILD groups. RESULTS: 226 patients were included, 36 meeting the IPAF criteria. IPAF patients demonstrated a high prevalence of autoantibodies to tRNA synthetase (35.3%), Ro52 (27.8%), and neutrophilic cytoplasmic antigens (ANCA; 20.0%). IPAF and CTD-ILD patients demonstrated similar clinical characteristics (mean age 66.6 and 63.7 years, respectively, female predominant, frequent CTD-manifestations). Lung function did not differ between ILD groups. Disease severity, pulmonary hypertension (PH), and ILD-MDM diagnosis were strong predictors of worse transplant-free survival (TFS). Meeting the IPAF criteria was not associated with TFS. CONCLUSIONS: We identified IPAF as a heterogeneous phenotype that overlaps considerably with CTD-ILD. Disease severity, PH, and ILD-MDM diagnosis were more powerful predictors of survival outcomes than meeting the IPAF criteria.

The supportive care needs of people living with pulmonary fibrosis and their caregivers: a systematic review.

BACKGROUND: People 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. METHODS: A 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. RESULTS: A 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. CONCLUSION: People 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.

Nonspecific Interstitial Pneumonia.

Nonspecific interstitial pneumonia (NSIP) is a complex disorder commonly associated with other conditions such as connective tissue diseases (CTDs) and environmental exposures. Although idiopathic NSIP has been recognized as a separate clinical entity, recent studies have suggested that a proportion of these cases have autoimmune features suggestive of underlying CTDs. The diagnosis of NSIP usually carries a better prognosis compared with idiopathic pulmonary fibrosis but has an unpredictable natural history. Its pathogenesis is thought to be an inflammatory-driven process involving multiple pathways, including a genetic predisposition. The lack of specific clinical features often makes the diagnosis of NSIP difficult. The huge variability of radiological and histological features seen in NSIP adds to the complexity of achieving an accurate diagnosis of NSIP and a multidisciplinary approach is often required. There is a lack of consensus on the optimal management strategy of NSIP. Early clarification of the goals of therapy and close monitoring for the progression of disease is important across the spectrum of NSIP irrespective of its etiology. Although immunosuppressive and immunomodulatory agents are commonly used for severe and progressive disease, the therapeutic landscape of NSIP is constantly evolving as the role of newer agents such as antifibrotic therapies is being explored.