Is There Relief for Patients With Interstitial Lung Disease?

December 18, 2022

Doctor pointing to scan of lungs in front of patient

Daniel Dilling, MD

Interstitial lung disease (ILD) includes a range of conditions that affect lung function.

Though some ILDs can be progressive and life-threatening, new research is improving care. Loyola Medicine is at the leading edge of this research.

“We cannot cure ILD yet,” says pulmonologist and Loyola Medicine medical director of lung transplantation Daniel Dilling, MD.

“But we can provide relief from symptoms and slow the progress of the disease over time.”

What Is Interstitial Lung Disease?

Inside your lungs, blood vessels surround small air sacs called alveoli. Between the blood vessels and air sacs is a membrane or wall, called the interstitium. This space is where oxygen and carbon dioxide get exchanged between blood and air. ILD affects the alveoli, interstitium and blood vessels.

“Interstitial lung disease is not a specific disease in itself,” says Dr. Dilling. “It’s a category of diseases that cause inflammation and scarring (fibrosis) of the interstitial tissue. The scarring prevents gas exchange.” Not all ILDs cause scarring. Those that do are called pulmonary fibrosis.

ILD can be mild, moderate or severe. Some types of ILD progress rapidly. Symptoms of ILD include:

Shortness of breath
Dry cough
Fatigue

What are the Types of Interstitial Lung Disease?

There are over 150 types of ILD. The main categories of ILDs include the following:

Autoimmune Disease-Related ILDs

Autoimmune disease-related ILDs are when your body attacks your lungs and causes scarring. Examples include rheumatoid arthritis, Sjögren’s syndrome and scleroderma.

Exposure-Related ILDs

Exposure-related ILDs are caused by substances in your environment such as dust, mold, bacteria, and plant and animal proteins.

Idiopathic ILDs

Idiopathic ILDs are a range of ILDs that have no known cause. Idiopathic pulmonary fibrosis is the most common type of idiopathic ILD and is the model of a progressive ILD.

Medical therapy-related ILDs

Medical therapy-related ILDs are caused due to radiation therapy or medications.

Sarcoidosis

Sarcoidosis is an inflammatory disease that causes tiny sites of inflammation (granulomas) to form in your lungs and other organs.

How Do I Know if I Have Interstitial Lung Disease?

Interstitial lung disease can be complex to diagnose. “Many studies have shown that people with ILD often have an incorrect diagnosis for months or years,” says Dr. Dilling. “They may think they have a different condition or the wrong type of ILD.”

Loyola offers second opinions to help patients get the correct ILD diagnosis and treatment plan. The initial appointment includes:

In-depth interview to identify past exposure to environmental agents
Physical exam, including pulmonary function testing
Review of all available medical records, including previous blood tests, imaging tests and biopsy results

Loyola’s multidisciplinary team of pulmonologists, radiologists and pathologists meets to evaluate each case. Through a collaborative discussion, the team comes to a consensus regarding the diagnosis and treatment plan.

“We usually see patients who have been to multiple pulmonologists before coming to us,” says Dr. Dilling. “It’s not uncommon to change a patient’s diagnosis, treatment plan or prognosis.”

Advances in Interstitial Lung Disease Treatment

Treatment of ILD, especially progressive forms such as idiopathic pulmonary fibrosis (IPF), has evolved over the past several decades.

In the early 2000s, the standard treatment for IPF was a triple therapy of prednisone, azathioprine and N-acetylcysteine (NAC).

To verify the effectiveness of triple therapy, the National Institutes of Health (NIH) funded a large trial called PANTHER. “The results were surprising — triple therapy was actually harming patients,” says Dr. Dilling. “After the trial ended, we entered a period of time where there was no standard treatment for IPF.”

Since then, two antifibrotic medications (nintedanib and pirfenidone) have emerged as effective treatments for slowing IPF progression. Dr. Dilling was a co-investigator on the ASCEND trial that showed pirfenidone was an effective treatment. This pivotal study led to FDA approval in 2014.

An international trial called INBUILD, which also included Dr. Dilling as a co-investigator, evaluated nintedanib for other types of progressive ILDs. “Now, if I have a patient with a progressive ILD that isn’t IPF, I can prescribe nintedanib,” says Dr. Dilling. “That wouldn't have been possible five years ago.”

Several other clinical trials at Loyola Medicine that offer people access to leading-edge therapies include:

PRECISIONS Trial: N-Acetylcysteine (NAC) Therapy for Idiopathic Pulmonary Fibrosis

Data analyses from the PANTHER trial found a benefit with NAC-only treatment in patients with a genetic variation of the TOLLIP gene. This finding led to a new study called PRECISIONS.

Loyola is one of about 25 sites for the PRECISIONS trial and is actively recruiting patients.

“About one in four people with IPF have the TOLLIP gene variation,” says Dr. Dilling.

“Whether these patients benefit from NAC therapy is an important question. Somewhere on the horizon, we might have personalized therapy for people with IPF based on their genes.”

STRIVE-IPF Trial: Treating Acute Idiopathic Pulmonary Fibrosis Exacerbations

People with IPF are at risk of acute exacerbations, or flare-ups, that often lead to hospitalization. Acute exacerbations do not respond well to treatment and are too often the cause of death in people with IPF.

Loyola is part of an NIH-sponsored trial (STRIVE-IPF) investigating a new set of therapies for acute exacerbation. “Preliminary data have shown extraordinary outcomes with this strategy,” says Dr. Dilling.

IMPALA-2 Trial: Use of Inhaled Molgramostim Nebulizer Solution in Autoimmune Pulmonary Alveolar Proteinosis (aPAP)

PAP is a rare lung disease with very limited treatment options. This inhaled medication is in a phase 3 clinical trial and brings hope of relief for these patients.

Loyola is one of a small group of medical centers asked to participate in this trial.

MILED Trial: Early Treatment of Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that usually affects women in their 30s and 40s. The cysts impair lung function and, over time, can lead to respiratory failure.

Sirolimus is the only approved treatment for LAM and only for people with moderate to severe disease.

“We are part of the MILED clinical trial, investigating low-dose sirolimus therapy for people with mild disease,” says Dr. Dilling. “This trial will help us understand whether it’s safe and effective to use sirolimus early on, rather than waiting for symptoms to progress.”

Loyola Medicine is a world-renowned LAM center and is part of the U.S. LAM Clinics and Research Network.

Dr. Dilling and Loyola pulmonologist Krishnan Warrior, MD also recently published a review article on lung transplantation for LAM in the prestigious Journal of Heart and Lung Transplantation.

Patient Registries

Patients can also participate in registries to help researchers understand ILD better. “A registry is a long-term data bank that stores clinical data and blood specimens for research,” says Dr. Dilling.

“We collaborate with other research institutions to study these diseases and push the science of ILD forward.”

Loyola participates in several important registry studies, including IPF/ILD PRO for fibrotic lung diseases, MIDAS for LAM and the Pulmonary Fibrosis Foundation Patient Registry.

Treating ILD with a Lung Transplant

When ILD treatments are no longer effective, lung transplant may be an option. Loyola opened one of the nation’s first lung transplant program in 1988 (the first in Illinois) and has completed nearly 1,200 transplants since.

“Lung transplant is a lifesaving surgery,” says Dr. Dilling. “We provide personalized care to help patients manage long-term immunosuppression and maximize their quality of life.”

Is Stem Cell Therapy an Effective Treatment for ILD?

Mesenchymal stem cell therapy is a controversial and unapproved treatment for ILD offered by several “for profit” clinics around the country.

Loyola’s new chair of the department of medicine, Marilyn Glassberg Csete, MD, is an internationally recognized researcher in the field of stem cell therapy for ILD.

“Patients often ask me about the value of stem cell therapy,” says Dr. Dilling. “Dr. Glassberg’s work has shown us that mesenchymal stem cells are not an effective treatment, though research is ongoing.”

Finding Relief for ILD at Loyola Medicine

U.S. News & World Report recognized Loyola Medicine among the top 10% in the nation for Pulmonary & Lung Surgery. Loyola is also part of the Pulmonary Fibrosis Foundation Care Center Network.

This expertise translates to positive results for patients. “I've had people with interstitial lung disease turn around their symptoms and bring their condition under control,” says Dr. Dilling.

“For patients with idiopathic pulmonary fibrosis, we may not be able to turn things around, but we can stabilize them and give them a better prognosis.”

Loyola’s pulmonology and critical care specialists also offer:

Pulmonary rehabilitation to help patients improve their lung function
Palliative care to help patients take control of a life-limiting illness

To make an appointment for a pulmonology evaluation or second opinion consultation, call 888-584-7888 or self-schedule an appointment online using myLoyola.

Daniel Dilling, MD, FACP, FAST, FCCP, is a pulmonologist and medical director of lung transplantation at Loyola Medicine. He is board certified in critical care medicine, pulmonary disease and internal medicine.

Dr. Dilling earned his medical degree at Loyola University Chicago Stritch School of Medicine and completed his internship and fellowship at Loyola University Medical Center.

Book an appointment today to see Dr. Dilling, another Loyola pulmonologist or thousands of other Loyola Medicine providers by self-scheduling an appointment online using myLoyola or by calling 888-584-7888.

Sources

American Lung Association — Lymphangioleiomyomatosis (LAM) — https://www.lung.org/lung-health-diseases/lung-disease-lookup/lymphangioleiomyomatosis-lam
NIH National Heart, Lung, and Blood Institute — Sarcoidosis — https://www.nhlbi.nih.gov/health/sarcoidosis
StatPearls — Ninedanib — https://www.ncbi.nlm.nih.gov/books/NBK585049/