• Emphysema is a
respiratory disease that
result in millions alveoli
stretching out of shape
• As the alveoli become
damaged or destroyed,
the lungs lose their
elasticity and become
unable to empty easily.
Combined Pulmonary Fibrosis and
• Combined pulmonary fibrosis and emphysema
is characterized by the coexistence of both
upper lobe emphysema and lower lobe
• Tobacco smoking is said to be the main factor
in its etiology.
• It is mostly seen in males older than 60 with a
substantial smoking history(> 40 year pack
• It is a unique form of idiopathic pulmonary
fibrosis because of the preserved lung volumes
due to the opposing effects of both emphysema
and fibrosis on the total lung volume.
• Patients classically present with progressive
shortness of breath, with symptoms lasting for
about 3 years before diagnosis.
• Hypoxemia is a common finding and it is
generally mild to moderate at rest and gets worse
• Physical examination usually reveals bilateral
lower lobe inspiratory crackles and digital
• Pulmonary hypertension is highly prevalent in
this disease and is the leading cause of death.
• Patients CPFE may have a higher prevalence of
lung cancer, with squamous cell carcinoma
being the most common histologic type.
• High-resolution computed tomogram is the
imaging of choice, and it typically shows the
characteristic upper lobe paraseptal
emphysema with bullae, and lower lobe
subpleural honeycombing with interstitial
fibrosis and traction bronchiectasis.
• Surgical lung biopsy is usually necessary to
make a confident clinicopathologic diagnosis
• Corticosteroids, immunosuppressive agents,
and antibiotic agents offer little to no benefit.
• Home oxygen can be offered for symptomatic
• Lung transplant has been shown to offer some
survival advantage in IPF patients.
• 70-year-old Hispanic male who presented with
progressive shortness of breath
• Said he was in his usual state of health until the
year before, when he started getting short of
breath with exertion
• Initially, dyspnea occurred only with severe
exertion, but it progressively got worse to the
point where the patient was getting short of
breath even with mild exertion and sometimes at
• Over the last year he also had a persistent dry
• Exercise tolerance had dramatically decreased,
to less than a block
• He denied any paroxysmal nocturnal dyspnea,
chest pain, fever, or hemoptysis
• 40 pack-year history, and he quit 3 years ago
• Past medical history included benign prostatic
• Denied any surgical history
• Denied any alcohol or drug abuse.
• He worked as a laborer all his life, including
occasional painting and marble cleaning jobs.
• Influenza and pneumococcal vaccines are up to
Vital signs and basic lab work were normal
Low oxygen saturation of 92%.
Inspiratory crackles and substantial clubbing
On 6-min walk test he walked only 200 m
(normal predicted was 600 m)and desaturated
• Peak expiratory flow was 550 L/min
• HRCT showed lower
with fibrosis and
• Bilateral upper lobe
• Pulmonary function tests showed no
restrictive or obstructive pattern
• Showed preserved lung volumes with severely
decreased diffusing capacity
• Echocardiogram showed moderate pulmonary
hypertension with right ventricular systolic
pressure of 50 mm Hg
• I was healthy a year ago , then I started
getting short of breath with exertion.
• Shortness of breath
• Vital signs and lab work: Normal with Spo2 at
• Dry cough
• Inspiratory crackles
• Peak expiratory flow 500L/min
• CXR:Bilateral lower lobe pulmonary fibrosis
• HRTC: lower lobe subpleural honeycombing,
fibrosis, traction bronchiectasis, and bilateral
upper lobe paraseptal emphysema with bullae
• PFT: No restrictive or obstructive pattern,
preserved lung volumes with severely
decreased diffusing capacity
• ECG: Moderate pulmonary hypertension with
RV systolic pressure of 50 mm Hg(normal <25