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Source publication
Interstitial lung diseases (ILD) are a diverse group of pulmonary diseases for which accurate diagnosis is critical for optimal treatment outcomes. Diagnosis of ILD can be challenging and a multidisciplinary approach is recommended in international guidelines. The purpose of this position paper is to review the evidence for the use of the multidisc...
Context in source publication
Context 1
... Guidelines from the American Thoracic Society and European Respiratory Society (ATS/ERS) recommend testing for antinuclear antibody (ANA), anti-cyclic citrulli- nated peptide (anti-CCP) and rheumatoid factor (RF) in all patients with suspected ILD. 36 Because the diagnosis of CTD-ILD has implications for treatment and prognosis, if there is any suggestion of CTD symptoms, a more detailed autoimmune screen can be performed (Table 3) and review by a rheumatologist or immunologist is advisable. Although blood tests alone are rarely diagnostic of a spe- cific ILD, certain autoantibodies are more specific for CTD such as anti-CCP for rheumatoid arthritis; dsDNA and anti-Smith antibodies for systemic lupus erythematosus; and anti-topoisomerase (Scl-70) for systemic sclerosis (scleroderma). ...
Citations
... Multidisciplinary discussion (MDD) can facilitate a timely and accurate diagnosis of ILD, incorporating input from various clinicians, radiologists, pathologists and other specialists. Although the 2022 ATS/ERS/JRS/ALAT guidelines [1] focus on MDD for IPF, there is recognition of the importance of MDD for other ILDs as well [32,33,40,41]. While the current study found that in just over 75% of cases no additional physicians were involved in the diagnosis, 83% of respondents reported being part of a multidisciplinary team, with most having a role in diagnosis and ongoing management. ...
Background
For patients with interstitial lung diseases (ILDs) presenting with a progressive pulmonary fibrosis (PPF) phenotype, current knowledge of disease characteristics at diagnosis, patient journey, and treatment is limited. This study aimed to describe demographics and clinical experiences of patients presenting with PPF in a European real-world setting.
Methods
Data were analysed from the Adelphi Real World PPF-ILD Disease Specific Programme™, a cross-sectional survey of pulmonologists and rheumatologists in five European countries (France, Germany, Italy, Spain, United Kingdom) and internal medicine specialists (France) from April to October 2022. Physicians provided data for up to 12 consecutive patients with physician-confirmed ILD with a progressive phenotype other than idiopathic pulmonary fibrosis. Analyses were descriptive.
Results
Overall, 265 physicians reported on 1,335 patients. Mean (standard deviation) age at survey date was 60.4 (11.6) years, 91.2% were white, 58.1% female, 44.0% non-smokers. Most patients (63.3%) first consulted a primary care physician. There was a mean delay of 7.8 (22.7) months between first ILD symptom and healthcare professional visit, and another 7.7 (12.8) months to ILD diagnosis. At survey date, 47.7% of patients had physician-reported moderate ILD, 42.3% had mild ILD and 10.0% had severe ILD. Disease progression was reported in the 12 months prior to the survey for 19.5% of patients; of these, progression was based on worsening symptom in 27.3% and lung function decline in 25.8%. For patients experiencing symptoms prior to ILD diagnosis (72.8%), the most common symptoms were dyspnoea on exertion (80.5%) and cough (57.8%). Overall, 17.4% of patients were misdiagnosed prior to ILD diagnosis, with chronic obstructive pulmonary disease suspected in 39.2% of them. The most frequent comorbidities were anxiety (16.9%) and gastroesophageal reflux (15.5%). Although 77.8% of patients were receiving treatment for ILD at survey date, 15.6% of patients had never been prescribed treatment for ILD.
Conclusions
This real-world study expands our understanding of patients, diagnostic delays and treatment gaps experienced by patients diagnosed with PPF in Europe. There was a mean delay of 15.5 months between first ILD symptoms and ILD diagnosis. Given the progressive nature of PPF, diagnostic delay may lead to poor outcomes, including shorter survival.
Trial registration
N/a.
... Best practice for ILD diagnosis mandates a multidisciplinary meeting of specialist clinicians, including respiratory physicians, pulmonary radiologists and pathologists [2]. Consensus diagnosis must balance detailed clinical assessment, examination findings, radiological assessment with high-resolution computed tomography of the chest, serological evaluation and, on occasion, bronchoalveolar lavage [2,3]. ...
... Best practice for ILD diagnosis mandates a multidisciplinary meeting of specialist clinicians, including respiratory physicians, pulmonary radiologists and pathologists [2]. Consensus diagnosis must balance detailed clinical assessment, examination findings, radiological assessment with high-resolution computed tomography of the chest, serological evaluation and, on occasion, bronchoalveolar lavage [2,3]. A significant minority of people with ILD will receive a diagnosis that is unclassifiable or made with low confidence, in which case lung biopsy may be recommended to increase diagnostic confidence, provide accurate disease prognostication and guide treatment decisions regarding antifibrotic and immunosuppressive pharmacotherapy [3][4][5]. ...
... Dense fibrosis, often associated with advanced ILD, is less favourable for histological assessment than milder fibrosis [51] and may contribute to the mechanism by which reduced lung function is associated with lower yield and pneumothorax. While undertaking an ILD multidisciplinary meeting after lung biopsy is known to increase diagnostic confidence [2], the positive impact on diagnostic yield of a meeting prior to cryobiopsy is novel. ILD discussion may promote selection of appropriate patients and exclusion of inappropriate candidates. ...
Background
Transbronchial lung cryobiopsy (TBLC) is an alternative to surgical lung biopsy for histopathological evaluation of unclassifiable interstitial lung disease (ILD) or ILD diagnosed with low confidence. This meta-analysis synthesised current literature regarding cryobiopsy diagnostic performance and safety, focusing on procedural and sampling techniques.
Methods
Medline and Embase were searched on 11 April 2022. Studies included adults with unclassifiable ILD, reporting diagnostic yield, complications and methodological techniques of TBLC. Meta-analyses were performed for diagnostic yield, pneumothorax and bleeding. Subgroup analyses and meta-regression assessed methodological variables. PROSPERO registration: CRD42022312386.
Results
70 studies were included with 6183 participants. Diagnostic yield of TBLC was 81% (95% CI 79–83%, I ² =97%), with better yield being observed with general anaesthesia (p=0.007), ILD multidisciplinary meeting prior to cryobiopsy (p=0.02), 2.4 mm cryoprobe (p=0.04), higher mean forced vital capacity (p=0.046) and higher mean diffusing capacity for carbon monoxide (p=0.023). Pneumothorax rate was 5% (95% CI 4–5%, I ² =91%), with higher rates associated with a 2.4 mm cryoprobe (p<0.00001), routine post-procedure imaging (p<0.00001), multiple lobe sampling (p<0.0001), reduced mean diffusing capacity for carbon monoxide (p=0.028) and general anaesthesia (p=0.05). Moderate-to-severe bleeding rate was 12% (11–14%, I ² =95%) and higher rates were associated with a 2.4 mm cryoprobe (p=0.001) and bleeding score selection (p=0.04).
Interpretation
Patient characteristics and modifiable factors, including procedural methods and anaesthetic techniques, impacted diagnostic yield and safety outcomes of TBLC in people with unclassifiable ILD and contributed to heterogeneity of clinical outcomes. These variables should be considered for individualised clinical decision making and guideline development and warrant routine reporting in future research.
... Interstitial lung diseases (ILD) are a heterogeneous group of disorders comprising more than 200 entities [1]. Although the clinical manifestations of ILD are similar in different entities, the corresponding therapeutic options differ, as well as their prognosis [2][3][4]. Therefore, there remains a need for precise and specific diagnostic methods to guide clinical management of this complex condition. In the current clinical practice, a reliable diagnosis of ILD can be established in the majority of cases by patient history, imaging and serologic analysis, followed by multidisciplinary discussion (MDD). ...
Rationale and objectives
Bleeding is a major complication of transbronchial lung cryobiopsy (TBLC), and pre-placing a bronchial balloon is one of the clinical practices used to prevent it, but with very weak evidence, which should be confirmed. This study aimed to conduct whether pre-placing a bronchial balloon in TBLC for diagnosing interstitial lung disease (ILD) is more safety.
Materials and methods
In this prospective, single-center, randomized controlled trial, patients with suspected ILD were enrolled and randomly assigned to pre-placed balloon and none-pre-placed balloon groups. The primary outcome was incidence of moderate bleeding in each group. The secondary endpoints were the incidence of severe bleeding, pneumothorax, and other procedural complications.
Results
Exactly 250 patients were enrolled between August 2019 and March 2022, with 125 in each group. There were no significant differences in severe bleeding between the none-pre-placed balloon group and pre-placed balloon group (1.6% vs. 0.8%; adjusted p = 0.520), while more moderate bleeding occurred in the none-pre-placed balloon group (26.4% vs. 6.4%, adjusted p = 0.001), as well as more use of hemostatic drug (28.0% vs. 6.4%, adjusted p = 0.001). Three patients in the none-pre-placed balloon group used the bronchial balloon. More samples could be acquired in the pre-placed balloon group than in the none-pre-placed balloon group (3.8 ± 0.9 vs. 3.1 ± 0.9, p < 0.001). There were no significant differences in multidisciplinary discussion (MDD) between the two groups (89.6% vs. 91.2%, adjusted p = 0.182).
Conclusion
A pre-placed bronchial balloon can reduce the incidence of moderate bleeding and increase the confidence of the bronchoscopists. However, it had no effect on increasing the diagnostic rate of MDD and reducing severe bleeding.
Registration number: NCT04047667 (www.clinicaltrials.gov identifier).
... Based on clinical suspicion, an MDT discussion may help in choosing the best biopsy method and in determining the most appropriate area for tissue sampling, so that the sample is histologically adequate to provide diagnostic information to pathologist. Particularly in institutions lacking expertise in TBLC, MDT discussion can guide the selection of patients for SLB or, in selected case, for TBB, which may be useful in the assessment of processes with a lymphangitic distribution, such as sarcoidosis or silicosis, or bronchiolocentric distribution, such as HP or OP [280]. Furthermore, pathologists can explain the histological report and eventually gene expression profile data in MTD discussion, in order to correlate their findings with those of other specialists and so establish the correct diagnosis [281]. ...
Idiopathic Interstitial Pneumonias (IIPs) are a heterogeneous group of the broader category of Interstitial Lung Diseases (ILDs), pathologically characterized by the distortion of lung parenchyma by interstitial inflammation and/or fibrosis. The American Thoracic Society (ATS)/European Respiratory Society (ERS) international multidisciplinary consensus classification of the IIPs was published in 2002 and then updated in 2013, with the authors emphasizing the need for a multidisciplinary approach to the diagnosis of IIPs. The histological evaluation of IIPs is challenging, and different types of IIPs are classically associated with specific histopathological patterns. However, morphological overlaps can be observed, and the same histopathological features can be seen in totally different clinical settings. Therefore, the pathologist’s aim is to recognize the pathologic–morphologic pattern of disease in this clinical setting, and only after multi-disciplinary evaluation, if there is concordance between clinical and radiological findings, a definitive diagnosis of specific IIP can be established, allowing the optimal clinical–therapeutic management of the patient.
... Although MDD is strongly recommended by international guidelines [7,15] a consensus approach for the management of MDD is still lacking. A tentative standardization of MDD has been proposed with the publication of the position statement by the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia [20]. ...
... The recent coronavirus (COVID-19) pandemic has led to the rapid spread of video conferencing technologies in MDD ILDs. Telemedicine has been applied in MDDs even before the COVID-19 pandemic: Australia has used a hybrid virtual/in-person MDD approach in its clinical practice [20]. Telemedicine exploits the use of advanced and secure platforms for data sharing, also allowing greater collaboration between specialists and different centers and may help in the standardization of ILD-MDM in the near future [47]. ...
Introduction
The diagnosis of Idiopathic pulmonary fibrosis (IPF) requires the careful exclusion of secondary causes of interstitial lung disease (ILD), and the collaboration among different specialists is considered paramount to establish a diagnosis with high diagnostic confidence. The multidisciplinary discussion (MDD) has assumed an increasing importance over the years in the different phases of the IPF diagnostic work-up.
Areas covered
The role of MDD in the diagnosis and management of IPF will be described. Practical insights will be provided into how and when to perform MDD based on the available scientific evidence. Current limitations and future perspectives will be discussed.
Expert opinion
In the absence of high diagnostic confidence, agreement between different specialists during MDD is recognized as a surrogate indicator of diagnostic accuracy. Often, despite a lengthy evaluation, the diagnosis remains unclassifiable in a significant percentage of patients. MDD therefore appears to be pivotal in attaining an accurate diagnosis of ILDs. The discussion among different specialists can also include other specialists, such as rheumatologists and thoracic surgeons, in addition to the core group of pulmonologists, radiologists, and pathologists. Such discussions can allow greater diagnostic accuracy and have important effects on management, pharmacologic therapies, and prognosis.
... Current international recommendations for IPF diagnosis, as well as the increasing number of published national recommendations, indicate the benefits of a multidisciplinary discussion (MDD) in the diagnostic process of every patient undergoing diagnostics due to an ILD of unknown etiology with signs of fibrosis and clinical suspicion of IPF [5,7,27,[45][46][47][48]. The consistency between a single-discipline decision (SDD), i.e., a decision established by one specialist or a group of physicians in one specialty, and an MDD is rated at 70% (47-87%) [49][50][51][52][53]. ...
The recommendations were developed as answers to previously formulated questions concerning everyday diagnostic and therapeutic challenges. They were developed based on a review of the current literature using the GRADE methodology. The experts suggest that PF-ILD be diagnosed based on a combination of different criteria, such as the aggravation of symptoms, progression of radiological lesions, and worsening of lung function test parameters. The experts recommend a precise diagnosis of an underlying disease, with serological testing for an autoimmune disease always being included. The final diagnosis should be worked out by a multidisciplinary team (MDT). Patients with an interstitial lung disease other than IPF who do not meet the criteria for the progressive fibrosis phenotype should be monitored for progression, and those with systemic autoimmune diseases should be regularly monitored for signs of interstitial lung disease. In managing patients with interstitial lung disease associated with autoimmune diseases, an opinion of an MDT should be considered. Nintedanib rather than pirfenidon should be introduced in the event of the ineffectiveness of the therapy recommended for the treatment of the underlying disease, but in some instances, it is possible to start antifibrotic treatment without earlier immunomodulatory therapy. It is also admissible to use immunomodulatory and antifibrotic drugs simultaneously. No recommendations were made for or against termination of anti-fibrotic therapy in the case of noted progression during treatment of a PF-ILD other than IPF. The experts recommend that the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation should be applied to patients with an interstitial lung disease other than IPF with progressive fibrosis as in patients with IPF.
... The MDM was first recommended to replace histopathology as the gold standard in the 2002 American Thoracic Society (ATS)/European Respiratory Society (ERS) Joint Statement on the Classification of Idiopathic Interstitial Pneumonias (4). This recommendation was re-emphasized in the 2013 update (1) as well as in more recent position statements (5,6) and clinical practice guidelines for the diagnosis of idiopathic pulmonary fibrosis (7,8), hypersensitivity pneumonitis (9) and progressive pulmonary fibrosis (8). ...
... Despite scarcity of supporting evidence, expert panels have suggested approaches to ILD MDM organization in attempts to provide a standard framework for universal implementation (5). As a minimum standard, these have recommended attendance by at least two respiratory physicians in addition to a radiologist and tissue pathologist, recognizing that this is clearly not feasible in every setting. ...
... Importantly, consensus should be achieved on whether there is a need for invasive testing with lung biopsy for each case (7,8). Additionally, international consensus guidelines on standardized diagnostic ontology for fibrotic ILDs have also recommended classification of expected disease behavior to help inform patient prognosis, treatment goals and future monitoring strategy (1,5,28). ...
The multidisciplinary meeting (MDM) has been endorsed in current international consensus guidelines as the gold standard method for diagnosis of interstitial lung disease (ILD). In the absence of an accurate and reliable diagnostic test, the agreement between multidisciplinary meetings has been used as a surrogate marker for diagnostic accuracy. Although the ILD MDM has been shown to improve inter-clinician agreement on ILD diagnosis, result in a change in diagnosis in a significant proportion of patients and reduce unclassifiable diagnoses, the ideal form for an ILD MDM remains unclear, with constitution and processes of ILD MDMs varying greatly around the world. It is likely that this variation of practice contributes to the lack of agreement seen between MDMs, as well as suboptimal diagnostic accuracy. A recent Delphi study has confirmed the essential components required for the operation of an ILD MDM. The ILD MDM is a changing entity, as it incorporates new diagnostic tests and genetic markers, while also adapting in its form in response to the obstacles of the COVID-19 pandemic. The aim of this review was to evaluate the current evidence regarding ILD MDM and their role in the diagnosis of ILD, the practice of ILD MDM around the world, approaches to ILD MDM standardization and future directions to improve diagnostic accuracy in ILD.
... Canadian Thoracic Society (2017) [90] • Should involve expert respirologists, radiologists and pathologists However, the scope of MDTs in ILD has expanded to encompass other specialists who may benefit the dynamic discussions around diagnosis, monitoring disease progression and management decisions. For example, rheumatologists and transplant surgeons are also key contributors in an MDT to provide management recommendations for CTD-ILDs and lung transplantation, respectively, although the availability of these speciality groups to participate in a meeting is a limitation [26]. Pulmonologists may be best placed to take part in monitoring and management discussions as they directly consult with the patient, along with integrating clinical data. ...
... Additional noncore members may also advise on the supportive care for patients (palliative care and transplant teams) but may not need to attend regular MDT meetings. Assessing patient comorbidities and preferences is especially important considering that treatment options are often associated with adverse events that may affect a patient's quality of life [26]. ...
... Poor inter-MDT agreement was found between different centres in diagnosing HP and nonspecific interstitial pneumonia, which may be related to the lack of diagnostic criteria for these ILD subtypes [10]. Further research is needed on shared practices among ILD MDTs, including the format of meetings, documentation of cases and meeting coordination, to reduce heterogeneity and allow for inter-MDT benchmarking [26,74]. A standardised template of collated patient data and regular multidisciplinary conferences (ideally once every 2 weeks) can increase harmonisation and maintain expertise [19,26,74]. ...
Multidisciplinary team (MDT) meetings, involving the integrated collaboration of healthcare professionals, are increasingly used in clinical practice to inform the diagnosis and treatment of interstitial lung diseases (ILDs). Over time, the assessment of patients with ILD has transitioned from discussions among clinicians, radiologists and pathologists to the inclusion of a broader range of clinical data and specialist expertise. Studies have shown that a multidisciplinary approach can have many benefits for the clinical care of patients with ILD by improving the diagnostic confidence for different ILDs and guiding treatment decisions. The utility of MDT discussions for diagnosis, monitoring disease progression and management decisions, will need to be considered based on how it is best positioned in the diagnostic and therapeutic process, as well as the practicality and challenges of its use. There are also uncertainties and heterogeneity concerning the optimal practices of MDT meetings in ILD care. In this review, we describe recent developments refining the approach to MDTs in clinical practice, including who should be involved in the MDTs, when it is most needed, their use in patient management, challenges in their implementation, and ongoing controversies in the field that need further research.
... Practice guidelines from multiple societies and international policy documents emphasize the importance of delivering equitable and appropriate palliative care to people diagnosed with advanced heart failure (HF) and chronic respiratory diseases, such as chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) [e.g., (1)(2)(3)(4)(5)]. People living with these progressive conditions will eventually experience physical function decline, as well as changes to their psychological, social and spiritual functioning and wellbeing (6)(7)(8). ...
... Test-retest reliability (64) Face, content validity (63, 65) Construct validity (64) Inter-rater reliability (64) NEST (6) Respect/support from health professionals (5) Meaning of life (6) Autonomy (7) Share emotions (2) Religious needs (2) Importance: 1 ("not at all important") to 5 ("very important") Satisfied: 1 ("not met") to 5 ("met in full") Higher score = greater importance/satisfaction ...
Background
The impetus to develop and implement tools for non-malignant patient groups is reflected in the increasing number of instruments being developed for heart failure and chronic respiratory diseases. Evidence syntheses of psychometric quality and clinical utility of these tools is required to inform research and clinical practice.AimsThis systematic review examined palliative care needs tools for people diagnosed with advanced heart failure or chronic respiratory diseases, to determine their: (1) psychometric quality; and (2) acceptability, feasibility and clinical utility when implemented in clinical practice.Methods
Systematic searches of MEDLINE, CINAHL, Embase, Cochrane and PsycINFO from database inception until June 2021 were undertaken. Additionally, the reference lists of included studies were searched for relevant articles. Psychometric properties of identified measures were evaluated against pre-determined and standard criteria.ResultsEighteen tools met inclusion criteria: 11 were developed to assess unmet patient palliative care needs. Of those, 6 were generic, 4 were developed for heart failure and 1 was developed for interstitial lung disease. Seven tools identified those who may benefit from palliative care and include general and disease-specific indicators. The psychometric qualities of the tools varied. None met all of the accepted criteria for psychometric rigor in heart failure or respiratory disease populations. There is limited implementation of needs assessment tools in practice.Conclusion
Several tools were identified, however further validation studies in heart failure and respiratory disease populations are required. Rigorous evaluation to determine the impact of adopting a systematic needs-based approach for heart failure and lung disease on the physical and psychosocial outcomes of patients and carers, as well as the economic costs and benefits to the healthcare system, is required.
... We retrospectively recruited patients with an acute exacerbation of ILD and viral infection, who were hospitalized between 1 January 2016 and 31 December 2019, at two secondary and tertiary academic hospitals in China. IPF was defined by the 2007 American Thoracic Society/ European Respiratory Society criteria [8]; the definition was broadened to include patients with previously known or established fibrotic disease at admission [9]. We enrolled patients who had usual interstitial pneumonia patterns on their radiological examination, meaning those with an acute exacerbation of connective tissue disease (CTD)-associated interstitial pneumonia and unilateral lung transplantation for ILD. ...
Introduction. Patients with interstitial lung disease (ILD) who subsequently develop a viral infection have high rates of morbidity and mortality.
Hypothesis/Gap Statement. Few large-scale epidemiological studies have investigated potential prognostic factors for morbidity and mortality in this patient group.
Aim. To evaluate the risk factors for morbidity and mortality in hospitalized patients with ILD and viral infection, as well as the clinical characteristics.
Methodology. This retrospective cohort study included patients with ILD who were hospitalized for a viral infection in two tertiary academic hospitals in China, between 1 January 2013 and 31 December 2019. We analysed the prevalence of comorbidities, clinical characteristics, 30 day mortality rates, and prognostic risk factors.
Results. A total of 282 patients were included; 195 and 87 were immunocompromised and immunocompetent, respectively. The most common underlying interstitial diseases were idiopathic pulmonary fibrosis (42.9 %) and connective tissue disease (36.9 %). The 30 day mortality rate was 20.6 %. During the influenza season, an increase in influenza virus (IFV) (25.7 %), respiratory syncytial virus (14.9 %) and cytomegalovirus (CMV) (11.3 %) cases was observed in the immunocompromised group. The most frequently detected virus in the immunocompetent group was IFV (44.8 %), followed by respiratory syncytial virus (11.5 %), and human rhinovirus (9.2 %). During the non-influenza season, CMV (34.4 %) was the main virus detected in the immunocompromised group. The 30 day mortality rates of non-IFV patients were higher than those of IFV patients. Older age (>60 years), respiratory failure, persistent lymphocytopenia, invasive mechanical ventilation and non-IFV virus infection were significantly associated with increased 30 day mortality.
Conclusion. Patients with ILD who develop viral infection have high rates of morbidity and mortality, which are associated with increased age (>60 years), respiratory failure, mechanical ventilation, persistent lymphocytopenia and non-IFV virus infection. These risk factors should be carefully considered when determining treatment strategies for this patient population.
