Many Misdiagnoses, Few Treatments for Idiopathic Pulmonary Fibrosis Patients

Vol. 13 •Issue 9 • Page 37
Many Misdiagnoses, Few Treatments for Idiopathic Pulmonary Fibrosis Patients

Clinicians Learn Ways to Counsel Patients Diagnosed With an Incurable Disease

Given three years to live, Michael Rosenzweig, PhD, has survived for four, walking two miles a day and refusing steroids, which he calls “as bad as the disease.”

His disease is idiopathic pulmonary fibrosis (IPF), a rare form of chronic fibrosing interstitial pneumonia. In IPF, a progressive scarring of tissue, with a tendency to start in the lower lobes, proceeds upward.

Symptoms progress gradually to dyspnea on exertion, nonproductive paroxysmal coughing, bi-basal crackles (described as a Velcro-like sound), reduced lung capacity, impaired gas exchange — and death, usually three to five years after diagnosis for an estimated two-thirds of patients.

It has no known cause or cure.

At the 2004 American Thoracic Society conference, Dr. Rosenzweig, 75, a retired Chicago psychologist, shared the emotional journey he’s taken since hearing his diagnosis, working through denial to depression to anger and, finally, to acceptance.

“Recognize that you will die, and start to prepare for it,” instructed Dr. Rosenzweig, who has lost two siblings to IPF. “The only problem is you don’t know when it will happen.”

The most common complaint he hears in talking to 50 or so IPF patients a week is one shared frequently in this era of managed care: The doctor won’t talk to me. “There’s really no effort to understand the deterioration of quality of life for patients who go through this,” said Dr. Rosenzweig, who co-founded the Pulmonary Fibrosis Foundation in 2000, a Chicago-based organization dedicated to funding IPF research.

The foundation has published a patients’ handbook and distributed copies to 13,000 pulmonologists. “The reception we’re getting is amazing,” he said. “There’s such a hunger out there for information.”

A 2004 survey of 1,200 IPF patients conducted by another group, the Coalition for Pulmonary Fibrosis (CPF), based in San Jose, Calif., echoed the same concern. A majority complained they weren’t well-informed about their disease.

“This was the primary reasoning for establishing the CPF in 2001,” said Mark Shreve, the CPF’s chief operating officer.

“There also remains a critical need for improved education of the health care professionals who treat the IPF patient, from the primary care physician to the pulmonologist on down to the critical care nurses and respiratory therapists, so that every patient is afforded the highest level of care possible.”

When counseling IPF patients, “you must offer an honest prognosis,” said John Hansen Flaschen, MD, chief of the pulmonary, allergy and critical care division of the University of Pennsylvania Medical Center in Philadelphia.

Median survival for IPF is three years, yet it’s hard to predict, he said. Predictors of short survival in IPF include severe functional impairment, low diffusion capacity, and the extent of fibrosis recorded by high-resolution computed tomography scans.

Ask patients if they’ve been thinking about how or when they might die. “Believe me,” Dr. Hansen-Flaschen assured ATS delegates, “they have thought about it.”

Be forthright. “Your lung reserve is so reduced now that you may die at any time,” is candid but truthful. Consider hospice care when the patient’s functional reserve becomes severely limited.

Encourage the patient to plan for death, but don’t convey the notion you’re giving up on him, Dr. Hansen-Flaschen suggested. “Hope and expect for the best, but prepare for the worst,” says it best.


Of the approximately 200 interstitial lung diseases, IPF is one of the rarer forms. However, the current estimate of 83,000 cases in the United States with 31,000 new cases surfacing each year probably lowballs reality, experts believe.

“I’ve heard estimates that perhaps as many as 20 percent of IPF patients are misdiagnosed,” Shreve said. “Meanwhile, the clock is ticking.”

The CPF’s survey found 55 percent of respondents were initially misdiagnosed with another pulmonary condition: 17 percent misdiagnosed with bronchitis, 13 percent with chronic obstructive pulmonary disease, 13 percent with an unspecified respiratory ailment, and 12 percent with asthma.

“Almost every person I’ve talked to with IPF has been misdiagnosed as having asthma, COPD or just that it was old age,” Dr. Rosenzweig said. “Then they finally see a specialist and get the real story.”

Even pulmonologists can face a daunting task rendering a definitive diagnosis, though. No marker is yet known for the diagnosis of IPF, according to James Dauber, MD, clinical director of the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease at the University of Pittsburgh.

In most cases, clinical and .high-resolution computed tomography evaluations are sufficient to diagnose IPF, he said, citing the results of a 2001 study.1 If they’re inconclusive, however, lung surgical biopsy is needed. “You need a large piece of lung tissue to make this diagnosis,” he said.


As for risk factors, most IPF patients are 50 or older, and 70 percent are current or former smokers. Male patients outnumber females. Heavy exposure to environmental irritants such as asbestos, pesticides, wood dust and metal dust are implicated, along with chronic aspiration associated with GERD.

Published data show that as many as 20 percent of IPF cases .may be genetically inherited. One CPF board member has lost five family members to the disease, Shreve said. “Research.ers have not identified a specific gene involved in IPF, but there seems to be a genetic predisposition to the disease in some families.”

Researchers at Duke University Medical Center are now collecting data on patients with two or more family members diagnosed with IPF. The four-year project, funded by the National Institutes of Health, will analyze blood samples from more than 850 family members in an effort to isolate a common defective gene.

Ongoing research like the Duke project was rare in 2000, when Dr. Rosenzweig co-founded the Pulmonary Fibrosis Foundation. “There was nothing going on in terms of research trying to understand the disease and find effective treatments for it,” he said. “That was a very intolerable situation from my point of view.”

Along with his brother, who has since died from IPF, and Marvin Schwarz, MD, a director and professor of pulmonary sciences and critical care medicine at the University of Colorado, Denver, Dr. Rosenzweig decided the time was ripe to “shake the bushes” for IPF research funding.

Now six pharmaceutical firms are testing IPF drugs with funding from the foundation, he said. In addition, the National Institutes of Health, which already has two studies ongoing, plans to spend at least $25 million on a national network for clinical research into IPF, he said.


To date, though, no treatment short of lung transplantation consistently alters the pattern suffered by most IPF patients: rapid decline in the first few years after diagnosis, then stabilization, then a gradual decline.

The Food and Drug Administration has yet to approve any treatment for the disease.

In the 1950s, believing it inflammatory in nature, clinicians prescribed prednisone, which entailed many side effects, Dr. Dauber said. The 1990s saw a switch to anti-oxidants, then to anti-fibrotic agents and, now, to immunomodulators.

Short a cure, current therapeutic strategies focus on how to control one of IPF’s major symptoms: dyspnea.

IPF patients suffer three types of dyspnea: dyspnea on exertion, which subsides when patients stop to rest; paroxysmal dyspnea; and persistent dyspnea at rest, which plagues patients who are actively dying.

“This is what patients most fear in the final stages of lung disease,” Dr. Hansen-Flaschen said. Most health care professionals, he added, are “bad at managing it.”

Pulmonary rehabilitation is recommended for dyspnea, offered Susan Jacobs, RN, MS, a clinical research nurse in pulmonary medicine at Stanford University Medical Center, Stanford, Calif. The rehab regimen should include aerobic exercise, pursed-lip breathing, strength and stretching training, relaxation techniques, music and other distractions, yoga, and opiates, she said.

The message for respiratory clinicians is: Add life to years rather than years to life.

That motto found its perfect image in a photograph Jacobs displayed of a patient of hers with severe IPF. The man had his heart set on just one goal: to climb back on his horse. Sure enough, there he sat astride his colt, proud in the saddle, portable oxygen at his side.


1. Hunninghake G, Zimmerman M, Schwartz D, et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Resp Crit Care Med. 2001;164:193-6.

2. Hope-Gill B, Hilldrup S, Davies C, et al. A study of the cough reflex in idiopathic pulmonary fibrosis. Am J Resp Crit Care Med. 2003;168:995-1002.

Michael Gibbons is senior associate editor of ADVANCE. Visit for more on IPF.