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Idiopathic Pulmonary Fibrosis: A Case Study, Approach to Diagnosis, and Research.
Maglaris, D, Jederlinic, P, MD.
Central Maine Medical Center, Lewiston, ME.
Introduction: IPF is a less common form of lung disease with an approximate prevalence of 14-42.7 per
100,000 in the US in 2006 or 26 per 100,000 in the European Union in 2012. Despite these numbers, the
life expectancy from the time of diagnosis is 2-3 years. When a patient presents with worsening
shortness of breath, it is worth doing a thorough work up, because often, this illness can be overlooked
and misdiagnosed as COPD. Once the diagnosis is made, the patient needs to be directed to treatment,
or research, because unfortunately, this disease has an ill-fated course.
Case: A 67-year-old male came to the pulmonary clinic for shortness of breath, which he had for a
number of years but seemed worse over the past 6 months. He was a 1.5 pack per day smoker for 48
years until he discontinued 7 years ago. He had a CT scan in 2009 showing abnormal lymph nodes but
had bronchoscopy at that time with a negative transbronchial needle aspiration. He has a history of
squamous cell carcinoma of the tongue treated surgically in 2009. He also underwent evaluation for
obstructive sleep apnea but was not currently being treated with CPAP, because he could not tolerate it.
He notes a shortness of breath occurs with one flight of stairs, sometimes less. He has an albuterol
inhaler, although he rarely uses it, because he does not think it helps much. He has no history of asthma
or seasonal allergies. He denies any cough, sputum, wheezing, chest pain, or syncope. He denies any
history of blood clots, hemoptysis, tuberculosis, or lung cancer. The patient was found on physical exam
to have a few crackles at the lung bases. A recent chest x-ray showed increased interstitial markings,
more so than in the film from 2010. His CT scan showed peripheral and basal interstitial lung disease
with honeycombing and a question of empysematous cysts. His most recent spirometry was normal. The
question is whether his CT changes are due to emphysema with fibrosis or an idiopathic pulmonary
fibrosis, given the worsening CT finding over a 3-year period. The next steps were to rule out connective
tissue disease, so RF, ACE, and ANA were ordered. The patient planned to return in 6 weeks and then
in 6 months for complete pulmonary function testing. For symptomatic relief he planned to lose weight.
Should this be IPF, any respiratory illness superimposed on the fibrosis will be treated. Should he want
more definitive treatment, a clinical study could be offered to him.
Discussion: Given this case, it is important to understand the stepwise diagnostic approach in lung
disease and the consideration of differential diagnoses each step of the way. Over the past decade, the
definition of IPF has grown more specific, differentiating itself from other ILD’s. The definition of IPF: a
chronic fibrosing interstitial pneumonia of unknown cause, with a histologic pattern of usual interstitial
pneumonia (UIP) on surgical lung biopsy. Although a lung biopsy is not critical, an appropriate substitute
would be high resolution CT image. Overall, four diagnostic criteria are required: 1. Exclusion of other
causes of ILD. 2. Abnormal pulmonary function tests or impaired gas exchange. 3. Bibasilar reticular
abnormalities with minimal ground glass opacities on HRCT. 4. Transbronchial lung biopsy or
bronchoalveolar lavage specimens without features to support alternative disease. Despite a more
specific diagnosis, the options for an effective treatment are still minimal. Historically, the approach had
been to use corticosteroids and other immune modulators such as azathioprine or cyclophosphamide to
reduce what was thought to be a reactive immune response in the lungs. However, as recent as 2008,
these modalities have proven to be ineffective or even detrimental in the outcome of IPF. Since then, the
recognition of increased fibroblasts in the lung tissue of IPF patients has refocused research, targeting
the fibrosis cascade, angiogenesis, hormonal influences, and alternative inflammatory pathways.
Although many trials have led to dead ends, the molecule pirfenidone, a pyridine with anti-inflammatory,
antioxidant, and anti-fibrotic properties, has been approved for treatment of IPF in the European Union.
This molecule alters the expression, synthesis and possibly the accumulation of collagen, and it inhibits
the recruitment, proliferation, and expression of extra cellular matrix cells.