Spectrum of interstitial lung diseases and their association with pulmonary hypertension

Tyagi, Richa; Kant, Surya; Verma, Sanjeev Kumar; Kushwaha, Ramesh Kumar; Kumar, Santosh; Garg, Rajiv; Verma, Ajay Kumar; Srivastava, Anand; Bajaj, Darshan Kumar; Wakhlu, Anupam; Parihar, Anit; Pradhan, Akshyaya; Jaiswal, Riddhi

doi:10.4081/monaldi.2021.2027

Cited by 3 publications

(2 citation statements)

References 37 publications

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“…[23,24] However, it should be kept in mind that bronchiectasis and ILD are on a spectrum with patients having mild and others having advanced disease. [25] Therefore, we cannot generalize this to each ILD and bronchiectasis patient.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics and outcomes of Group 3 and Group 4 Pulmonary Hypertension

Salahuddin¹,

Shahid²,

Tariq³

et al. 2022

Preprint

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Introduction: Pulmonary hypertension (PH) is associated with increased mortality, and lung diseases are the second most common cause of PH. Transthoracic echocardiogram (TTE) is often employed for PH diagnosis in low-to-middle income countries. Methods: A retrospective chart review of adult patients hospitalized from June 2012 to May 2021. Patients with pulmonary artery systolic pressure (PASP) ≥ 35 mmHg on TTE were included in the study. Patients with group 1 PH, group 2, or group 5 PH were excluded. Results: 128 patients were included, with females being 69.5%. Group 3 PH consisted of 79.7% and Group 4 PH consisted of 20.3%. Obesity hypoventilation syndrome / obstructive sleep apnea (OHS/OSA) consisted of 31.3% of the population and was the most common etiology of PH. The overall 1-year mortality after diagnosis of PH was 20.3%, with bronchiectasis having the highest group-specific 1-year mortality of 45.5%. 1-year hospital readmission was seen in 46.9% of patients. The difference in mortality rates between the groups was statistically significant (x2 = 18.1, P=0.004). The mean PASP was 59.2 ± 16.5 mmHg. On multivariate analysis, patients who did not have 1-year hospital readmission or were treated with diuretics, had decreased odds of 1-year mortality. Conclusion: Clinical outcomes of patients with group 3 PH vary based on causative etiology. Patients with bronchiectasis had the highest 1-year mortality and 1-year readmission rate. Currently, TTE based diagnosis for PH is a reality in LMIC.

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Section: Discussionmentioning

confidence: 99%

Clinical characteristics and outcomes of Group 3 and Group 4 Pulmonary Hypertension

Salahuddin¹,

Shahid²,

Tariq³

et al. 2022

Preprint

View full text Add to dashboard Cite

show abstract

“…Similarly, PH was confirmed by RHC in 44% in a cohort of 50 chronic hypersensitivity pneumonitis (HP) patients ( 9 ). A 2021 cross-sectional study performed at a tertiary care center in India, showed 46% of 239 ILD patients (32.2% HP, 31.4% CTD-ILD, 36.4% other ILD diseases) had screening echocardiograms suggestive of PH (mPAP estimate > 20 mm Hg) at the time of diagnosis ( 10 ). Patients with CPFE are especially susceptible to development of PH with a prevalence of 30–50% ( 11 ).…”

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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Clinical significance of pulmonary hypertension in interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's innovative drug development initiative—Group 3 pulmonary hypertension

et al. 2022

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Pulmonary hypertension (PH) has been linked to worse outcomes in chronic lung diseases. The presence of PH in the setting of underlying Interstitial Lung Disease (ILD) is strongly associated with decreased exercise and functional capacity, an increased risk of hospitalizations and death. Examining the scope of this issue and its impact on patients is the first step in trying to define a roadmap to facilitate and encourage future research in this area. The aim of our working group is to strengthen the communities understanding of PH due to lung diseases and to improve the care and quality of life of affected patients. This introductory statement provides a broad overview and lays the foundation for further in‐depth papers on specific topics pertaining to PH‐ILD.

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Spectrum of interstitial lung diseases and their association with pulmonary hypertension

Cited by 3 publications

References 37 publications

Clinical characteristics and outcomes of Group 3 and Group 4 Pulmonary Hypertension

Clinical characteristics and outcomes of Group 3 and Group 4 Pulmonary Hypertension

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

Clinical significance of pulmonary hypertension in interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's innovative drug development initiative—Group 3 pulmonary hypertension

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