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Advancing care for idiopathic pulmonary fibrosis

Treating interstitial lung diseases requires a multidisciplinary approach

Division of Pulmonary, Critical Care and Sleep Medicine - May 2018



Associate Program Director, Division of Pulmonary, Critical Care and Sleep Medicine, UH Cleveland Medical Center; Clinical Assistant Professor of Medicine, Case Western Reserve University School of Medicine

Interstitial lung disease (ILD) is an umbrella term for more than 100 types of lung diseases that cause scarring and lack of elasticity in the lace-like network of tissues in the lungs. Known causes include exposure to organic or inorganic dusts (e.g., asbestos) and connective tissue diseases, such as lupus and Sjogrens. Idiopathic pulmonary fibrosis (IPF) is the most prevalent type of idiopathic interstitial pneumonia, a subgroup of ILD.

IPF is a complex disease and therefore demands a multidisciplinary team approach to interpret and integrate clinical, radiological and pathological data, explains Maroun Matta, MD, Associate Program Director in the Division of Pulmonary, Critical Care and Sleep Medicine at University Hospitals Cleveland Medical Center.

In fact, according to the 2011 international guidelines1 for the management of IPF, “Careful exclusion of alternative etiologies through multidisciplinary discussion between pulmonologists, radiologists, and pathologists experienced in the diagnoses of ILD is of the utmost importance to an accurate diagnosis.” The guidelines also suggest that when specialty centers run ILD multidisciplinary teams, it improves clinical outcomes.

“All members of the UH ILD multidisciplinary team contribute to patient care,” Dr. Matta says. “We work together to find the best care plan for each patient.”

High-resolution CT scans accurately diagnose 50 to 60 percent of cases of IPF.2

“If a CT scan is consistent with one type of injury or another, we don’t need a biopsy,” Dr. Matta explains. “However, in a subset of patients who don’t have obvious exposure, the CT scan can look like a variety of things. We don’t have a clear answer based on imaging, so the only option is to obtain a biopsy.”

Traditional bronchoscopic biopsies don’t yield big enough sample for diagnosis and video-assisted surgical biopsy expose the patient to surgical risks. Cryobiopsy, which uses a probe to freeze tissue, combines the benefits of bronchoscopy with the larger yield of surgical biopsy, and is now being offered at the UH Pulmonary Clinic.

“We get similar results with cryobiopsy; and patients are not admitted to the hospital and they don’t have the complications of surgical biopsy,” Dr. Matta says.

Idiopathic pulmonary fibrosis is the most lethal type of ILD, with a life expectancy of only three-to-five years. Until a few years ago, there were no active treatments for IPF except oxygen when needed and supportive care, Dr. Matta notes.

In October 2014, the Food and Drug Administration approved two treatments that slow the progression of IPF: Ofev (nintedanib) and Esbriet (pirfenidone). This has created a lot more interest in IPF research, Dr. Matta says. Now there’s upward of 100 studies in the pipeline.

Dr. Matta says the field of IPF is heading toward combination treatments. Most likely, any new drugs developed will be administered in combination with Ofev or Esbriet, as most patients will already be on one, or both, of these drugs. “In five years, things will look very different for these patients,” he says.

The UH Pulmonary Clinic is preparing to enroll patients in multiple studies for IPF and other ILDs.

The association between IPF and both gastroesophageal reflux disease (GERD) and obstructive sleep apnea (OSA) has been controversial, Dr. Matta notes. That is changing.

Studies have shown that GERD is both common and often asymptomatic in patients with IPF and the two diseases share risk factors, including age, smoking and being male.

“We’re making advances in understanding how acid coming from the stomach can lead to scar tissue in the lungs,” Dr. Matta says. This can occur even when patients are undergoing treatment for acid reflux.

“We screen all IPF patients for GERD and treat aggressively,” he says.

Similarly, studies show that 80 to 85 percent of patients with IPF also have sleep apnea, so the UH Pulmonary Clinic now screens patients for sleep apnea.

“An added advantage in our clinic is the opportunity to work closely with a sleep doctor if a patient does have sleep apnea,” Dr. Matta says. “In fact, a sleep specialist can be part of a patient’s multidisciplinary team.”


To refer your patients to the multidisciplinary IPF clinic, call (216) 844-2707. UH staff will coordinate all the required information for a successful patient visit.



  1. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. American Thoracic Society Documents. American Journal of Respiratory and Critical Care Medicine. VOL 183 2011.
  2. The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review. European Respiratory Review. 2015 24: 69-77.