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Interstitial Lung Disease: How This Common Lung Disorder Is Treated

Interstitial Lung Disease (ILD) is a group of lung disorders characterised by inflammation and scarring of the interstitium, the tissue surrounding the air sacs in the lungs. This condition can be challenging to diagnose and treat due to its diverse causes and symptoms. However, advancements in medical science have provided various treatment options to manage and alleviate its effects. 

Talking to the team of Onlymyhealth Dr Kinjal Modi, Consultant, Pulmonology, PD, Hinduja Hospital & MCR, Khar shared the complexities of ILD and explore the treatment modalities available to patients.

Treating Interstitial Lung Disease

Before delving into treatment, it's crucial to understand what ILD entails. "Interstitial Lung Disease encompasses a broad spectrum of conditions, including idiopathic pulmonary fibrosis, sarcoidosis, and hypersensitivity pneumonitis, among others," said Dr Modi. These diseases share common features such as inflammation and scarring of lung tissue, leading to impaired gas exchange and respiratory function.

Dr Modi listed a few ways to treat ILD:

Medications

The first step towards treating ILD is through medication:

Corticosteroids: These anti-inflammatory drugs are often prescribed to reduce inflammation in the lungs and alleviate symptoms.

Immunosuppressants: Medications like azathioprine, cyclophosphamide, and mycophenolate mofetil are used to suppress the immune system's abnormal response in certain ILDs.

Antifibrotic drugs: Pirfenidone and nintedanib are FDA-approved medications for treating IPF. They help slow down the progression of fibrosis, the hallmark of ILD.

Oxygen Therapy

Many ILD patients experience reduced oxygen levels in their blood due to impaired lung function. Oxygen therapy can help alleviate symptoms like shortness of breath and fatigue by providing supplemental oxygen.

Pulmonary Rehabilitation

This comprehensive programme includes exercise training, education, and psychological support to improve lung function, increase exercise tolerance, and enhance the overall quality of life for ILD patients.

Also Read: Respiratory Disease: What Is The Best Treatment For Asthma?

Lung Transplantation

For individuals with advanced ILD who do not respond to other treatments, lung transplantation may be considered as a last resort. This procedure involves replacing one or both diseased lungs with healthy donor lungs.

Clinical Trials

Researchers are continuously investigating new therapies and treatment approaches for ILD through clinical trials. Participation in these trials offers eligible patients access to cutting-edge treatments and contributes to advancing medical knowledge in the field.

Challenges In Treating Interstitial Lung Disease

While significant progress has been made in the treatment of ILD, several challenges persist. One major hurdle is the heterogeneity of ILD subtypes, which necessitates personalised treatment approaches tailoured to each patient's specific condition and underlying cause. Additionally, the long-term efficacy and safety of current treatments need to be further evaluated through ongoing research.

Looking ahead, emerging therapeutic strategies, such as targeted therapies and gene-based treatments, hold promise for improving outcomes in ILD patients. Furthermore, a better understanding of the underlying mechanisms driving lung fibrosis and inflammation will pave the way for the development of more effective and targeted therapies.

Also Read: Respiratory Diseases: Know Symptoms, Causes, Major Types And Preventions

Interstitial Lung Disease poses significant challenges to patients and healthcare providers alike, but with a multidisciplinary approach and ongoing research efforts, significant strides have been made in its management and treatment. From medications and oxygen therapy to pulmonary rehabilitation and lung transplantation, a range of options are available to help alleviate symptoms, slow disease progression, and improve the quality of life for individuals living with ILD. As research continues to unravel the complexities of this condition, the future holds promise for more targeted and effective treatment approaches, offering hope to patients and their families worldwide.

Disclaimer

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She Had Been Coughing For Two Years. The Cause Was A Surprise.

The voice on the phone was crisp and efficient. The 75-year-old woman listened, speechless. Her much-anticipated surgery to repair the crumpled vertebra in her lower back was canceled. The caller informed her that her chest X-ray, a routine pre-procedure test, was abnormal. She should see her doctor, and when her X-ray was normal, she could reschedule the operation. She summoned a polite response and hung up the phone. She could feel her heart racing and sat down.

She had had a cough for two years. It started not long after the pandemic descended on New York City and down the length of Long Island, even into the sleepy village of Quogue where she had moved to at the end of a busy career in Manhattan. After hacking away for several weeks — longer than any normal cough should last — she went to see her primary-care doctor. The doctor immediately tested her for Covid and sent her for an X-ray. When both were normal, doctor and patient were reassured. Her doctor suggested she get rid of her feather pillows and down comforter, in case they were causing an allergic reaction, and she did. It didn't help. Occasionally she considered going into the city to see a pulmonologist but knew the doctors there would be drowning in Covid cases. People were dying, and all she had was a cough.

But a year and a half later, she still had the cough. It was her constant companion, with her all day long, and according to her daughter — who moved back in with her when the pandemic struck — it was there all night as well. And it was a disgusting-sounding cough, as if she was coughing up something awful. Even worse, over the past couple of months she noticed that exercise was harder. She used to be a big walker — she and her dog could stride for miles on the beach and through the village. Now even a short walk could leave her out of breath. Not gasping but winded in a way she had never been from simply walking. Sometimes just making her bed would make her breathe a little harder than she should. Sure, she was 75, but she was a healthy 75. At least she thought she was.

It was when she noticed that she'd lost weight — more than 20 pounds — that she became worried. That's when she went to see an ear, nose and throat specialist, who examined her and put a camera down her throat. Everything looks fine, he told her. He thought it could be reflux and prescribed a medication for heartburn. When that didn't help either, she just let it go.

But now, just a few months later, this routine X-ray showed that there was something wrong with her lungs. She called her primary-care doctor once more. The doctor looked at the X-ray and immediately ordered a CT scan of the woman's chest. The results were posted in her electronic medical record within a day. The patient scanned them anxiously. She didn't understand a lot of the language, though it was clear the scan wasn't normal. She used the internet to try to understand what she was reading. Each page she read led to more searches and newer and stranger terms. Did she have interstitial lung disease? And exactly what was that? Finally, she found a sentence that put an end to her search: "The most common form of interstitial lung disease, idiopathic pulmonary fibrosis (I.P.F.), has a life expectancy of approximately three to five years."

Terrified, she called her old doctor, who had taken care of her for years when she lived in New York City. "Am I going to die in three to five years?" she asked her, as calmly as she could. She read the CT scan results to the doctor. She too was concerned by the radiologist's findings and suggested the patient see a pulmonologist. She knew a good one: Dr. Lester Blair at Weill Cornell Medicine in New York City. The older woman thanked her, then called his office immediately. She got an appointment for the following week.

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If Biopsy Needed For ILD Diagnosis, Start With Less-Invasive Option, Trial Suggests

When a biopsy is needed to diagnose interstitial lung disease (ILD), starting with a less-invasive approach can reduce patient burden while still achieving a diagnostic yield similar to surgical lung biopsy (SLB), a randomized trial suggested.

The step-up approach -- where patients underwent a transbronchial lung cryobiopsy and SLB was reserved only for cases where more information was needed for diagnosis -- significantly reduced the need for chest tube drainage when compared with immediate SLB, which requires hospital admission for post-surgical drainage.

For the study's primary endpoint, unexpected chest tube drainage in the step-up arm occurred in 11% of patients, while 46% of patients in the immediate SLB arm required prolonged chest tube drainage (P=0.0058), reported researchers led by Kirsten Kalverda, MD, of Amsterdam University Medical Centers.

Furthermore, patients assigned to the step-up strategy had shorter in-hospital stays compared with immediate SLB (median 1 vs 5 days, respectively), fewer serious adverse events (4% vs 50%), and less pain, according to findings in Lancet Respiratory Medicine.

"A multidisciplinary team discussion, resulting in a consensus diagnosis, is considered the reference standard for diagnosing ILD," explained Kalverda and colleagues. "In most cases, a high-confidence diagnosis can be established from clinical and radiological data, yet it is estimated that in 10-40% of newly diagnosed ILD, a lung biopsy is deemed necessary to establish a diagnosis or improve diagnostic confidence."

Lung tissue sampling via SLB has been considered the gold standard and is the most commonly used method, though it is associated with high morbidity and even mortality. Transbronchial cryobiopsy, meanwhile, has more recently become an alternative, though it has a lower diagnostic yield.

In the study, diagnostic yield reached 82% with transbronchial cryobiopsy alone, 88% with immediate SLB, and 89% with the step-up strategy.

"Recently published guidelines from the European Respiratory Society suggest using transbronchial lung cryobiopsy in patients with undiagnosed ILD in whom a histological diagnosis is indicated and who are eligible for SLB," wrote Margaret Salisbury, MD, of Vanderbilt University Medical Center in Nashville, Tennessee, and Kevin Flaherty, MD, of the University of Michigan in Ann Arbor, in an invited commentary.

"This recommendation was based on a balance between a lower rate of mortality and other serious complications against a lower performance on diagnostic outcomes," they continued. "The guideline committee also suggested using a step-up SLB after a non-diagnostic transbronchial lung cryobiopsy."

But, said Salisbury and Flaherty, no randomized trial supported these conditional recommendations.

As to the study results, they pointed out that some participants in the step-up arm should have gone on to SLB but did not, which could have affected the primary outcome. "It is also probable that many classifying diagnoses were made with low confidence," they added.

Of the 28 patients initially assigned to the less-invasive approach, only three of the five without a classifying diagnosis went on to SLB following the cryobiopsy.

Treatment selection in ILD -- a heterogeneous group of more than 10 disorders -- relies on identifying a specific diagnosis for proper management, the editorialists noted. But in the step-up group, just 46% of pathologists were able to make a diagnosis with high confidence as compared with 74% for the immediate SLB group.

"Although this first-in-class randomized comparison of two valid diagnostic strategies for ILD provides important data in this field, we continue to walk a fine line balancing procedural complications against diagnostic accuracy," the editorialists concluded.

The COLD study randomized 55 patients from six hospitals in the Netherlands to either the step-up approach (n=28) or immediate SLB (n=27). Patients were eligible for the study if their clinical and radiological data were insufficient to establish a classifying ILD diagnosis, and if multidisciplinary team discussion indicated obtaining lung tissue for diagnosis.

For the primary outcome, unexpected chest tube drainage in the step-up group was defined as any chest tube after transbronchial cryobiopsy while prolonged chest tube drainage after SLB was defined as drainage lasting more than 24 hours. Patients, pathologists, and clinicians were not masked to treatment assignment.

The average patient age was 66 years, 81% were men, and about three-fourths were former smokers. Following the biopsies, final diagnoses included hypersensitivity pneumonitis in 55%, idiopathic non-specific interstitial pneumonia in 13%, idiopathic pulmonary fibrosis in 8%, sarcoidosis in 4%, unclassifiable in 12%, and other in 12%.

Overall, adverse events occurred in 61% of patients in the step-up group and 75% of those in the immediate SLB group. Air leak, pneumonia, and uncontrolled pain were the most common serious adverse events.

Kalverda and colleagues noted that the small sample size and non-masked design may limit the study findings. Other potential limitations included that the large proportion of hypersensitivity pneumonitis cases, some differences in baseline measurements, and that all patients underwent intubated surgical procedures.

Elizabeth Short is a staff writer for MedPage Today. She often covers pulmonology and allergy & immunology. Follow

Disclosures

This study was supported by funding from the Netherlands Organisation for Health Research and Development and Amsterdam University Medical Centers.

No disclosures were reported by the study authors.

Salisbury reported relationships with the NIH, Boehringer Ingelheim, Orinove, and Roche. Flaherty reported relationships with the Pulmonary Fibrosis Foundation, Boehringer Ingelheim, Roche/Genentech, Bellerophon, Respivant, Shionogi, DevPro, AstraZeneca, Pure Health, Horizon, FibroGen, Sun Pharmaceuticals, Pliant, United Therapeutics, Arrowhead, Lupin, Polarean, PureTech, Trevi, CSL Behring, Daewoong, DisperSol, Immunet, NeRRe Therapeutics, Insilco, Vicore, GSK, Merck, and Chugai.

Primary Source

The Lancet Respiratory Medicine

Source Reference: Kalverda KA, et al "Transbronchial cryobiopsy followed by as-needed surgical lung biopsy versus immediate surgical lung biopsy for diagnosing interstitial lung disease (the COLD study): a randomised controlled trial" Lancet Respir Med 2024; DOI:10.1016/S2213-2600(24)00074-2.

Secondary Source

The Lancet Respiratory Medicine

Source Reference: Salisbury ML, Flaherty KR "Optimising the approach to ILD diagnosis: the balance of procedural complications against diagnostic accuracy" Lancet Respir Med 2024; DOI:10.1016/S2213-2600(24)00113-9.

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