Pulmonary hypertension in interstitial lung disease: an area of unmet clinical need

Dhont, Sebastiaan; Zwaenepoel, Bert; Vandecasteele, Els; Brusselle, Guy; Pauw, Michel De

doi:10.1183/23120541.00272-2022

Cited by 13 publications

(6 citation statements)

References 47 publications

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“…Prevalence of PH in ILD varies widely in the studies, based on population studied and how PH was defined. The prevalence has been reported as high as 86%, however, this estimate varies between different types of ILD ( 6 ). The majority of prevalence data is derived from idiopathic pulmonary fibrosis (IPF), the most common ILD.…”

Section: Epidemiology: a Common Consortmentioning

confidence: 99%

Evolution of pulmonary hypertension in interstitial lung disease: a journey through past, present, and future

Arslan,

Smith,

Qureshi

et al. 2024

Front. Med.

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Interstitial lung diseases (ILD) are a spectrum of disorders often complicated by pulmonary hypertension (PH) in its course. The pathophysiologic mechanism of WHO group 3 PH is different to other forms of PH. The advent of PH is a harbinger for adverse events like mortality and morbidity, implying that the PH component of disease expedites deteriorated clinical outcomes. In fact, WHO group 3 PH due to ILD has the worse prognosis among all groups of PH. Hence, early detection of PH by a comprehensive screening method is paramount. Given considerable overlap in clinical manifestations between ILD and PH, early detection of PH is often elusive. Despite, the treatment of PH due to ILD has been frustrating until recently. Clinical trials utilizing PAH-specific pulmonary vasodilators have been ongoing for years without desired results. Eventually, the INCREASE study (2018) demonstrated beneficial effect of inhaled Treprostinil to treat PH in ILD. In view of this pioneering development, a paradigm shift in clinical approach to this disease phenotype is happening. There is a renewed vigor to develop a well validated screening tool for early detection and management. Currently inhaled Treprostinil is the only FDA approved therapy to treat this phenotype, but emergence of a therapy has opened a plethora of research toward new drug developments. Regardless of all these recent developments, the overall outlook still remains grim in this condition. This review article dwells on the current state of knowledge of pre-capillary PH due to ILD, especially its diagnosis and management, the recent progresses, and future evolutions in this field.

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Section: Epidemiology: a Common Consortmentioning

confidence: 99%

Evolution of pulmonary hypertension in interstitial lung disease: a journey through past, present, and future

Arslan,

Smith,

Qureshi

et al. 2024

Front. Med.

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“…Pulmonary hypertension secondary to ILD has been associated to reduced exercise capacity, increased need for supplemental oxygen, worse quality of life and prognosis ( 230 – 232 ). Screening for pulmonary hypertension should be performed in all RA-ILD patients although no recommendation on timing and frequency is available ( 233 ). Recently, the INCREASE trial ( 234 ) has shown significant improvements in exercise capacity in ILD patients with PH treated with inhaled treprostinil.…”

Section: The Current Clinical Practice In the Management Of Ra-ildmentioning

confidence: 99%

Unmet needs and perspectives in rheumatoid arthritis-associated interstitial lung disease: A critical review

et al. 2023

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Rheumatoid arthritis (RA) is a chronic systemic autoimmune disease characterized by synovitis as the most common clinical manifestation, and interstitial lung disease (RA-ILD) represents one of the most common and potentially severe extra-articular features. Our current understanding of the mechanisms and predictors of RA-ILD is limited despite the demonstration that an early identification of progressive fibrosing forms is crucial to provide timely treatment with antifibrotic therapies. While high resolution computed tomography is the gold standard technique for the diagnosis and follow-up of RA-ILD, it has been hypothesized that serum biomarkers (including novel and rare autoantibodies), new imaging techniques such as ultrasound of the lung, or the application of innovative radiologic algorithms may help towards predicting and detecting early forms of diseases. Further, while new treatments are becoming available for idiopathic and connective tissue disease-associated forms of lung fibrosis, the treatment of RA-ILD remains anecdotal and largely unexplored. We are convinced that a better understanding of the mechanisms connecting RA with ILD in a subgroup of patients as well as the creation of adequate diagnostic pathways will be mandatory steps for a more effective management of this clinically challenging entity.

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“…63 Regardless the initial inciting triggers, idiopathic pulmonary fibrosis (IPF) and some other fibrosing ILD are characterized by progressive fibrosis of the lung interstitium, sharing a common final pathway leading to an irreversible, and self-perpetuating profibrotic process. 64 65 The most extensive available data on PH associated with ILD refers to IPF. However, many other forms of ILD, including chronic hypersensitivity pneumonitis, connective tissue disease (CTD)-related ILD, sarcoidosis, and pulmonary Langerhans cell histiocytosis are also commonly associated with PH, although its prevalence varies greatly depending on the underlying disease.…”

Section: Pulmonary Hypertension In Interstitial Lung Diseasementioning

confidence: 99%

“…However, many other forms of ILD, including chronic hypersensitivity pneumonitis, connective tissue disease (CTD)-related ILD, sarcoidosis, and pulmonary Langerhans cell histiocytosis are also commonly associated with PH, although its prevalence varies greatly depending on the underlying disease. 65…”

Section: Pulmonary Hypertension In Interstitial Lung Diseasementioning

confidence: 99%

Management of Pulmonary Hypertension Associated with Chronic Lung Disease

Blanco

Hernández-González

García

et al. 2023

Semin Respir Crit Care Med

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Pulmonary hypertension (PH) is a common complication of chronic lung diseases, particularly in chronic obstructive pulmonary disease (COPD) and interstitial lung diseases (ILD) and especially in advanced disease. It is associated with greater mortality and worse clinical course. Given the high prevalence of some respiratory disorders and because lung parenchymal abnormalities might be present in other PH groups, the appropriate diagnosis of PH associated with respiratory disease represents a clinical challenge. Patients with chronic lung disease presenting symptoms that exceed those expected by the pulmonary disease should be further evaluated by echocardiography. Confirmatory right heart catheterization is indicated in candidates to surgical treatments, suspected severe PH potentially amenable with targeted therapy, and, in general, in those conditions where the result of the hemodynamic assessment will determine treatment options. The treatment of choice for these patients who are hypoxemic is long-term oxygen therapy and pulmonary rehabilitation to improve symptoms. Lung transplant is the only curative therapy and can be considered in appropriate cases. Conventional vasodilators or drugs approved for pulmonary arterial hypertension (PAH) are not recommended in patients with mild-to-moderate PH because they may impair gas exchange and their lack of efficacy shown in randomized controlled trials. Patients with severe PH (as defined by pulmonary vascular resistance >5 Wood units) should be referred to a center with expertise in PH and lung diseases and ideally included in randomized controlled trials. Targeted PAH therapy might be considered in this subset of patients, with careful monitoring of gas exchange. In patients with ILD, inhaled treprostinil has been shown to improve functional ability and to delay clinical worsening.

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Pulmonary hypertension in interstitial lung disease: an area of unmet clinical need

Cited by 13 publications

References 47 publications

Evolution of pulmonary hypertension in interstitial lung disease: a journey through past, present, and future

Evolution of pulmonary hypertension in interstitial lung disease: a journey through past, present, and future

Unmet needs and perspectives in rheumatoid arthritis-associated interstitial lung disease: A critical review

Management of Pulmonary Hypertension Associated with Chronic Lung Disease

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