For the second annual Interstitial Lung Disease Day, Dr. Briana DiSilvio expands on the background of ILDs and the latest care practices.
Interstitial lung disease (ILD) emcompasses a myriad of inflammatory diseases that result in the scarring of lung tissue. Individuals with ILD typically experience strained breathing which interferes with the body’s ability to deliver oxygen to the bloodstream adequately.
In addition to respiratory distress, these individuals may present with dry cough, fatigue and weakness, discomfort in the chest, “clubbing” of the fingertips, loss of appetite and weight loss, according to the Pulmonary Fibrosis Foundation (PFF).
This year, an emphasis on awareness and recognition for the disease, and those living with it, is being observed on September 14, as the second annual Interstitial Lung Disease Day to occur, thanks to the collaborative effort of 9 organizations: Pulmonary Fibrosis Foundation, Arthritis Foundation, Foundation for Sarcoidosis Research, The Myositis Association, PF Warriors, Scleroderma Foundation, Scleroderma Research Foundation, Sjögren's Foundation, and Wescoe Foundation for Pulmonary Fibrosis.
With more than 200 possible causes, and over 50,000 new cases of ILD each year, healthcare professionals and advocacy organizations stress the ongoing need for expanded understanding, not only for affected patients, but healthcare providers as well. ILD Day serves as an opportunity to share resources and express support for those living with the disease or within the community.
In honor of ILD Day, Briana DiSilvio, MD, Allegheny Health Network (AHN) provided HCPLive with insight on the group of respiratory conditions. As a specialist in pulmonary and critical care, she has extensive experience treating patients with chronic lung conditions, pleural disease and respiratory infections, among others, with a clinical interest in pulmonary fibrosis and sarcoidosis.
HCPLive: Can you share some background on interstitial lung disease?
DiSilvio: Interstitial lung disease (ILD) is a term applied to a heterogeneous group of diffuse lung disorders which result from damage to the pulmonary parenchyma by ongoing inflammation. The end result of such repetitive injury is often pulmonary fibrosis and irreversible scarring.
ILDs are probably more appropriately classified under the category “diffuse parenchymal lung diseases (DPLDs)” as the inflammation and fibrosis tend to extend beyond the interstitial bed to involve the alveoli, alveolar ducts, and bronchioles. DPLDs can be broadly grouped into the following 6 categories: idiopathic (without a known etiology), autoimmune-related, environmental/occupational exposures, granulomatous lung disease, drug-induced.
Certain DPLDs, such as Idiopathic Pulmonary Fibrosis (IPF) and those associated with autoimmune diseases (e.g. Rheumatoid Arthritis- ILD, Systemic Sclerosis- ILD, Polymyositis/Dermatomyositis-ILD), are more likely to demonstrate a progressive phenotype with an often rapid decline in lung function and increasing extent of fibrosis over time. High resolution CT chest (HRCT) can offer a better characterization of the disease and even aid in diagnosis in cases of a normal appearing chest x-ray.
While surgical lung biopsies may be definitive in establishing a diagnosis, they are frequently not needed when classic radiologic patterns are present on HRCT. In large academic institutions with well-established ILD centers, a multidisciplinary approach involving pulmonologists, rheumatologists, radiologists, pathologists, and cardiothoracic surgeons can increase the accuracy of an ILD diagnosis while avoiding risk associated with invasive, and possibly unnecessary, procedures.
HCPLive: A new quantitative systems pharmacology (QSP) software was just released, how might this development, or others, improve the field?
DiSilvio: The mainstay of treatment for many DPLDs involves glucocorticoids (GCs) and immunosuppressive therapies (e.g. prednisone, mycophenolate mofetil, rituximab). Indications to use GCs and immunosuppressive agents depends on the primary disease, systemic activity, degree of active inflammation in the lungs, potential for reversibility, and overall ILD clinical course. Once DPLDs have entered a chronic phase of lung injury characterized by fibrosis, architectural distortion, traction bronchiectasis, and occasionally honeycombing, GCs and immunosuppressive therapies may be less beneficial.
In these instances, patients may benefit from anti-fibrotic therapies, only 2 of which are FDA approved–nintedanib and pirfenidone. The caveat to utilization of these medications is that they do not reverse the existing fibrosis but rather, only slow the progression of disease. Unfortunately, anti-fibrotics can have an intolerable side effect profile for many patients which often leads to cessation of therapy.
Quantitative systems pharmacology software certainly plays a role in helping to guide biomedical experiments that yield more meaningful data towards the development of newer therapies targeting ILDs, especially those with a progressive and fibrosing phenotype.
Briana DiSilvio, MD, Associate Program Director, Pulmonary Critical Care Fellowship, Co-Director, Autoimmune-ILD Clinic, Director of Fellowship Research, Associate Medical Director, AGH Pulmonary Function Lab, Division of Pulmonary, Critical Care, Sleep, and Allergy, Allegheny Health Network.