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• W2762343454 abstract "See related Article, See related Article Interstitial lung diseases (ILD) are a rare and heterogeneous group of lung disorders, with idiopathic pulmonary fibrosis (IPF) being the deadliest among them. Given the clinical impact of these diseases and their relative low prevalence, they have been the subject of a vigorous medical and scientific debate over the last years. In this context, guideline documents produced by scientific societies worldwide play a critical role. The two position papers endorsed by the Thoracic Society of Australia and New Zealand (TSANZ) and the Lung Foundation of Australia (LFA)1, 2 are focusing on two critical linked issues: the role of the multidisciplinary approach in the diagnosis of ILD and the treatment of IPF. These are crucial topics for clinical practice, since diagnosis and treatment are the building blocks of any disease: they have been initially combined in the 2011 ATS/ERS/JRS/ALAT guidelines,3 and then updated in 2015 for the treatment component.4 We are eagerly waiting for the update of the diagnostic part of these guidelines, which will provide a comprehensive, integrated approach to IPF patients. In the meantime, these Australian documents provide a solid, updated support to the management of patients with IPF. They will be used by many physicians, although differing legislation and rules in different countries may pose some challenges to the broader applicability of the recommendations in the guidelines. However, the positive cooperation between a scientific society, the TSANZ, and the pulmonary charity LFA allowed the production of clear and pragmatic documents. These two position papers have been written based on a comprehensive literature review and, when evidence was considered to be lacking, experts provided their opinion. This means that these documents cannot be considered strictly evidence-based (and some experts will consider this as a positive). LFA also provided the initial funding for the Australian IPF Registry, which represents a unique example of a high-quality registry, prospectively collecting critical data from the whole country. The methodology adopted by this Registry is based on a careful validation of the diagnosis in every single patient, through a process based on the multidisciplinary meeting (MDM). Therefore, the fact that each case is submitted to a central MDM before inclusion in the registry is assuring that the data generated apply to a homogeneous population of patients with IPF. We are sure that the data generated by such high-quality registry will help advance knowledge about the disease and inform future guidelines. The MDM approach proposed in the diagnostic document2 is well organized and based on modern communication tools, thus allowing the participation and the education of as many physicians as possible, even if living far from the central location of the Registry. Through this methodology, while assuring an expert approach to the diagnosis of each case, all MDM settings are allowed to participate and to grow locally. Ultimately, the aim will be to create a network of expertise, ensuring a similar and accurate diagnostic approach to each patient in Australia and New Zealand. This example will hopefully be implemented in other geographic areas dealing with similar logistic and operational challenges. The position paper on IPF treatment1 provides some food for thought. As in other geographic areas, there is some inhomogeneity in the management of IPF patients: for example, nintedanib is still not available in New Zealand. Also, there are differences in the prescription rules of disease-modifying drugs, oxygen and pulmonary rehabilitation, thus making the application of current international evidence-based guidelines difficult.4 Nonetheless, the document precisely reports the differences between Australia and New Zealand, with the aim of transparently presenting areas where controversy exists. As such, it is somehow surprising that in New Zealand the public funding for pirfenidone is discontinued if there is evidence of disease progression, defined as a forced vital capacity (FVC) decrease of >10% in any 12 months period. This is in line with the current UK guidelines,5 although recently Nathan et al.6 showed that continuing therapy with pirfenidone, even after the ‘progression’ of the disease, still reduced the risk of death and further FVC decline. Another area of uncertainty is the initiation of therapies in patients with mild, early disease. Wisely, the current document suggests an individualized approach to these patients, considering multiple factors, such as symptoms, age, co-morbidities and the impact of potential side effects. Considering the different methodologies used to produce the evidence-based international guidelines and the Australian position papers, can we spot any major, substantial difference? The answer is no. There are substantially homogeneous recommendations about nintedanib and pirfenidone, pulmonary rehabilitation and lung transplantation. A minor, but meaningful, difference is that in the Australian position paper there is no recommendation about the use of anti-acid medical or surgical therapies, while in the international guidelines the anti-acid medications received a conditional recommendation for use. As we know, this is still an area of great uncertainty7 and the Australian recommendation seems to be on the safe side. Finally, this position paper has stressed the importance of non-pharmacological interventions, which usually have very low quality of evidence, but are essential to assure the best possible quality of life for IPF patients. These include oxygen supplementation, treatment of co-morbidities, such as sleep-disordered breathing and emphysema, psychosocial support and palliative care. Last but not the least, these documents are a pleasure to read and provide an accessible although accurate basis for the best management of these challenging patients." @default.
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• W2762343454 date "2017-10-04" @default.
• W2762343454 modified "2023-10-18" @default.
• W2762343454 title "Managing patients with interstitial lung disease: Two more pieces of the puzzle" @default.
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• W2762343454 doi "https://doi.org/10.1111/resp.13195" @default.
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