Your patient who is experiencing chronic breathless could have idiopathic pulmonary fibrosis (IPF)—are you aware of the early signs? Eric S. White, MD, and Rebecca Weaver, APRN, have provided a case-based study to help illustrate the intricacies of this chronic disease that often first presents in the primary care office.
A 58-year old Caucasian male presented to his primary care physician (PCP) complaining of a bad cough, fatigue, and exertional breathlessness that started about 10 days prior. He describes several episodes of “bronchitis” and decreased energy level over the last year such that he is no longer able to play golf. He works at an exotic pet shop and has a 20 pack-year smoking history but quit five years ago. He denies chest pain or palpitations and attributes his symptoms to being out of shape. Physical examination showed normal vital signs, and lung sounds were unremarkable. Chest X-ray showed no infiltrate. He was started on antibiotics for suspected chronic bronchitis. Following the course of antibiotics, he began to feel better.
Three months later, the patient returned with worsening dyspnea on even modest exertion. Lung examination revealed mild bibasilar inspiratory crackles. Office spirometry was suggestive of a mild restrictive ventilatory defect. Due to his exposure to exotic animals, he was prescribed an inhaled corticosteroid/long-acting beta agonist for presumed occupational asthma.
After eight weeks on the inhaler regimen, the patient noticed no improvement and felt more dyspneic, even at rest. In the office he was noted to have hypoxemia and he was referred to a pulmonologist. The pulmonary consultant recommended a chest HRCT scan, which revealed basilar honeycombing, traction bronchiectasis, and reticulations, all consistent with a usual interstitial pneumonia (UIP) pattern. Serologic studies showed no evidence of an autoimmune disease or a hypersensitivity reaction.
The patient’s case was presented at a multidisciplinary interstitial lung diseases (ILD) discussion conference and he was diagnosed with IPF. The patient was started on antifibrotic therapy.
IPF is a rare disease that causes lung scarring1. Patients with IPF have a poor prognosis, with a median survival of two to five years2. The opportunity for earlier diagnosis of IPF exists at a PCP visit. A delay in diagnosis of IPF may lead to ineffective management3.
In this case study, there are several clues to a possible IPF diagnosis. At the PCP level, a high index of suspicion for IPF in patients with gradual progression of dyspnea, cough, and dry, inspiratory basilar (Velcro®) crackles4,5 is paramount. Knowledge of common risk factors for IPF can help a PCP identify IPF. Risk factors for IPF include age greater than 50, history of smoking, and male gender4,6.
IPF is diagnosed when an HRCT or lung biopsy demonstrates a UIP pattern and other causes of a UIP pattern are excluded7. In this case study, the HRCT is consistent with IPF and serologic data excludes autoimmune-associated ILD and hypersensitivity pneumonitis. The distinction is important, as autoimmune diseases and hypersensitivity pneumonitis may require alternative therapeutic approaches.
IPF is commonly initially mistaken for COPD, asthma, bronchitis, or CHF8. Misdiagnosis can lead to frequent physician visits, repeated diagnostic tests, exposure to harmful interventions, and delay in initiating appropriate treatment9. Thus, early diagnosis is critical in improving a patient’s prognosis9.
For more information, please visit the Pulmonary Fibrosis for Helathcare Professionals website or the Ohio Academy of Family Physicians’ (OAFP) Partner in Health web page and click on the Boehringer Ingelheim link. Boehringer Ingelheim, a leader in respiratory medicine for 100 years, is a proud Partner in Health of the OAFP. Partners in Health is an online resource that contains links to organizations that provide patient education and high-value tools to support family physicians and their care teams deliver optimal front line care.
Dr. White is a board-certified pulmonologist and a director in Clinical Development and Medical Affairs at Boehringer Ingelheim Pharmaceuticals, Inc. Dr. White was a professor of pulmonary and critical care medicine at the University of Michigan, where he specialized in the treatment of patients with interstitial lung diseases such as IPF. Ms. Weaver is a director in Clinical Development and Medical Affairs at Boehringer Ingelheim, focusing on interstitial lung diseases with the goal of improving the lives of patients. Previously, Ms. Weaver worked as a family nurse practitioner in South Florida.
- Meltzer EB and Noble PW. Orphanet J Rare Dis. 2008;3:8.
- Nathan SD et al. CHEST. 2011;140(1):221-229.
- Lamas DJ et al. Am J Respir Crit Care Med 2011;184(7):842-7.
- Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
- Raghu G et al. Clin Chest Med. 2004;25(3):409-419.
- Olson AL et al. Eur Respir Rev. 2018;27(150):180077.
- Raghu G et al. Am J Respir Crit Care Med. 2018;198(5):e44-e68.
- Zibrak JD et al. NPJ Prim Care Respir Med. 2014;24:14054.
- Cottin V et al. Eur Respir Rev. 2018;27(150):180076.