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
Lung Ultrasound Can Accurately Detect ILD In Patients With RA
The meta-analysis of 9 studies showed high sensitivity with slightly lower specificity with the less-invasive option for detecting lung complications in patients with the autoimmune disease.
An ultrasound of the lungs is a reliable, radiation-sparing, and cost-effective option for detecting lung complications in patients with rheumatoid arthritis (RA), according to a compilation of available data.1 The meta-analysis, detailed in Rheumatology & Autoimmunity, showed that interstitial lung disease (ILD) was accurately detected in patients through lung ultrasound, regardless of symptomatic or asymptomatic disease.
Ultrasound may be a more viable option than other methods, such as chest radiography and spirometry, to detect lung complications in rheumatoid arthritis.Image credit: Kate - stock.Adobe.Com
The data suggest that lung ultrasound is a more viable option than other methods, such as chest radiography and spirometry. They also mirror those observed among patients with systemic sclerosis, citing a meta-analysis that showed a sensitivity rate of 94% and lower specificity of 64%.
Within RA, a previous study published in 2024 indicated that lung ultrasound was a useful detection method for ILD when used in combination with diffusing capacity of the lungs for carbon monoxide, though researchers of that study cautioned against substituting HRCT with the method. There were 192 patients included in this study, among whom researchers found that B-lines over 11.5 on the ROC curve were predictive of ILD.2
The current meta-analysis describes the findings from 845 patients across the 9 studies, which showed a high sensitivity rate (91%; 95% CI, 0.837-0.953) with slightly lower specificity (79.3%; 95% CI, 0.509-0.934). Area under the curve (AUC) was 0.926.
"Most studies in our meta-analysis used the detection of B-lines in LUS as the criterion for ILD diagnosis, although other sonographic features, such as pleural irregularity and subpleural nodules, have also been reported," described the researchers. "B‐lines are vertical, laser‐like hyperechoic artifacts extending from the pleural line, indicative of pulmonary interstitial syndrome. These lines arise due to increased interstitial space or partial lung de-aeration, as seen in cases of pulmonary edema or ILD."
Eight of the 9 studies used B-lines as a criterion for determining ILD, among which the researchers found a similar sensitivity rate of 90.6% (95% CI, 0.826-0.952) and specificity rate of 73.9% (95% CI, 0.439-0.911). The AUC was 0.916.
A smaller analysis included 5 studies that assessed B-lines in 14 LIS points. This subgroup analysis showed similar sensitivity and specificity rates, with a sensitivity rate of 89.1% (95% CI, 0.746-0.958) and specificity of 89.1% (95% CI, 0.612-0.977). AUC for these studies was 0.938.
Two of the studies assessed 72 LIS, which improved sensitivity (91.7%; 95% CI, 0.798-0.968) but reduced specificity (56.4%; 95% CI, 0.440-0.682).
"While scanning more LIS has demonstrated good sensitivity, it is time-consuming and may not be practical for daily clinical use. Simplified methods evaluating fewer LIS, targeting sites strongly associated with ILD, have shown acceptable accuracy," wrote the researchers. "However, direct comparisons of LUS diagnostic accuracy based on the number of LIS examined are lacking, particularly in RA patients."
Existing research has pointed to a positive correlation between the amount of B-lines on the ultrasound and high-resolution computed tomography scores, though the researchers of this latest analysis struggled to pinpoint the optimal cutoff for lung ultrasound.
The cutoff for the number of B-lines used to diagnose the lung complication varied across the 8 studies. Studies that used a threshold of 5 B-lines led to a comparable sensitivity rate (94.8%; 95% CI, 0.834-0.985) but lowered the specificity rate to 60.4% (95% CI, 0.140-0.935), while a threshold of 10 B-lines led to both reduced sensitivity (88.1%; 95% CI, 0.778-0.939) and specificity (54.3%; 95% CI, 0.452-0.631).
"In conclusion, our meta-analysis suggests that LUS is a valuable tool for detecting ILD in patients with RA, demonstrating high overall diagnostic accuracy," the authors wrote. "As a noninvasive, radiation-free imaging modality, LUS can be safely and repeatedly used to identify lung abnormalities in RA patients that may warrant further evaluation with HRCT, facilitating timely diagnosis and early intervention. However, there remains a need for a validated scoring system and standardized protocols for LUS application in assessing RA-ILD."
References
Exploring Treatment Options For ILD
Interstitial lung disease (ILD) refers to a group of lung disorders that cause inflammation and scarring of lung tissue, leading to progressive breathing difficulties. While there is currently no cure for ILD, treatment focuses on controlling symptoms, slowing disease progression, and improving quality of life. If left untreated, ILD can lead to serious complications and early mortality. Management strategies typically include both pharmacological and non-pharmacological approaches, tailored to the specific ILD subtype and disease severity.
There currently is no cure for ILD and therefore management of the disease is mainly focused on controlling symptoms and slowing disease progression. Improperly treated ILD is associated with a poorer quality of life, an increased risk of life-threatening complications and early mortality [1]. The treatment of ILD usually involves a combination of both pharmacological and non-pharmacological approaches but this can vary based on the ILD subtype and disease severity.
Pharmacological
Those available pharmacological drug treatments for ILD can broadly be divided into three main categories; anti-inflammatory medications, immunosuppressants and antifibrotic medications. The treatment of ILD initially involves corticosteroids, to reduce inflammation and immunosuppressants to inhibit or reduce the immune response. Immunosuppressants are often given to reduce the amount of steroids the patient is taking particularly when the side effects are not well tolerated and consequently they are sometimes referred to as "steroid-sparing" medications. Additionally, anti-fibrotic medications are commonly given to ILD patients early in the disease course to slow the development of lung fibrosis.
Non-pharmacological
A variety of different non-pharmacological options also exist for ILD including smoking cessation, pulmonary rehabilitation, oxygen therapy, and lung transplantation.
Smoking Cessation
Smoking is a known causative or risk factor in several subtypes of ILD. For example, a recent study reported that current or former smokers were almost 60% more likely than lifelong nonsmokers to develop IPF [2]. However, limited evidence exists for the effects of smoking cessation on ILD outcomes although a few small studies have reported radiological improvements and the stabilization of ILD symptoms [3–6]. Regardless, ILD patients who continue to smoke put themselves at risk of developing other lung conditions such as COPD and lung cancer. Therefore, it is recommended that ILD patients undertake smoking cessation treatment in an effort to stop smoking.
Pulmonary Rehabilitation
Pulmonary rehabilitation describes a program of exercise training and disease education designed especially for those with chronic lung disease who suffer from persistent breathlessness. Evidence suggests that pulmonary rehabilitation can provide both short and long term improvements in functional exercise capacity, dyspnoea and quality of life in those with ILD [7]. However, pulmonary rehabilitation is not always widely available and may not be suited for all ILD patients i.E. Those with unstable cardiac disease or suffering from neuromuscular conditions.
Oxygen Therapy
Oxygen therapy is often prescribed in ILD patients who experience hypoxia at rest or on exertion. In fact estimates suggest that as many as 40% of ILD patients at some point require supplemental oxygen [8]. However limited evidence exists for the benefits of oxygen therapy on ILD outcomes although a few studies have reported that it can improve quality of life and exercise parameters in ILD patients for which hypoxia is a limiting factor [8]. The main limitation of oxygen therapy is the requirement for bulky gas cylinders which may limit its utility in improving exercise capacity.
Lung Transplant
Lung transplantation of either one of both lungs may be considered in ILD patients with severe disease who display a poor response to pharmacological treatments. Some common criteria for lung transplantation include an age below 70 and demonstrate a >10% reduction in FVC over 6 months and a <89% SaO2 or the need for
oxygen at rest [9]. Evidence suggests that lung transplantation can improve survival in carefully selected ILD patients [10]. However, there is a shortage of donor organs meaning one in six patients may die before a suitable donor is identified [9]. It is important to note that lung transplantation is a complex procedure that carries a significant risk of perioperative morbidity and mortality.
Managing ILD requires a multifaceted approach, combining medications, lifestyle changes, and in some cases, advanced interventions like lung transplantation. While there is no cure, ongoing research continues to enhance available therapies, offering hope for improved outcomes. If you or a loved one are facing ILD, seeking early and specialised medical care can help optimise treatment and enhance quality of life.
References
[1] Singh P, Ali SN, Zaheer S, et al. Cellular mechanisms in the pathogenesis of interstitial lung diseases. Pathol Res Pract [Internet].
2023;248:154691. Available from: https://www.Sciencedirect.Com/science/article/pii/S0344033823003916.
[2] Baumgartner KB, Samet JM, Stidley CA, et al. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1997;155:242–248.
[3] Mogulkoc N, Veral A, Bishop PW, et al. Pulmonary Langerhans' Cell Histiocytosis. Chest. 1999;115:1452–1455.
[4] Portnoy J, Veraldi KL, Schwarz MI, et al. Respiratory Bronchiolitis-Interstitial Lung Disease. Chest. 2007;131:664–671.
[5] Shinohara T, Kadota N, Hino H, et al. Improvement in idiopathic nonspecific interstitial pneumonia after smoking cessation. Respir Med Case Rep. 2015;14:7–9.
[6] Von Essen S, West W, Sitorius M, et al. Complete Resolution of Roentgenographic Changes in a Patient with Pulmonary Histiocytosis X. Chest [Internet]. 1990;98:765–767. Available from: https://www.Sciencedirect.Com/science/article/pii/S0012369216321237.
[7] Dowman L, Hill CJ, May A, et al. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database of Systematic Reviews. 2021;2021.
[8] Clark KP, Degenholtz HB, Lindell KO, et al. Supplemental Oxygen Therapy in Interstitial Lung Disease: A Narrative Review. Ann Am Thorac Soc. 2023;20:1541–1549.
[9] Wallis A, Spinks K. The diagnosis and management of interstitial lung diseases. BMJ. 2015;350:h2072–h2072.
[10] Chambers DC, Cherikh WS, Harhay MO, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-sixth adult lung and heart–lung transplantation Report—2019; Focus theme: Donor and recipient size match. The Journal of Heart and Lung Transplantation. 2019;38:1042–1055.
Lung Disease Trends In The UK, Before And After The COVID-19 Pandemic
Asthma, chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) affect more than 10 million people in the United Kingdom (England, Wales, Scotland and Northern Ireland), or approximately 15% of the population, but researchers believe a substantial number of diagnoses were missed during the COVID-19 pandemic.
A first-of-its-kind study headed by Hannah Whittaker, an inflammation and immunity research fellow at Imperial College London, aimed to show the burden of asthma, COPD and ILD in the U.K. Over a 20-year period using harmonized electronic health records to pave the way for federated data analyses across the country.
The study, published in Thorax on April 8, 2025, utilized the health data from across England, Wales, Scotland and Northern Ireland to examine trends in the incidence and prevalence of asthma, COPD and ILD between 2004 and 2023.
Before the pandemic, new cases of asthma and COPD had decreased across most of the U.K., while the incidence of ILD significantly increased.
The study uncovered a significant dip in lung disease diagnoses during the COVID-19 pandemic. Comparing expected to actual rates, Whittaker and her colleagues estimated that only about a third of anticipated new asthma and COPD cases were diagnosed, and ILD diagnoses were nearly 50% lower than expected. This suggests a large number of people with respiratory issues were likely undiagnosed and untreated.
The researchers concluded that the incidence of asthma decreased, the incidence of ILD increased and the incidence of COPD has decreased only in England and Wales.
"Changes in incidence rates were likely due to changes in behaviors and disease awareness," they wrote. "Disparities in incidence rates exist by sex, age, region, IMD (index of multiple deprivation) and ethnicity, and missed diagnoses from the COVID-19 pandemic could have a great impact on the future health of people with chronic respiratory diseases."
The study found a long-term decline in asthma and COPD incidence in the U.K. Before the pandemic, a significant increase in ILD incidence and a concerning drop in diagnoses for all three conditions during the pandemic, indicating a backlog of undiagnosed cases.
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