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Pulmonary Fibrosis
Pulmonary Fibrosis
DEFINITION
Idiopathic pulmonary fibrosis is defined as
a specific form of chronic, progressive
fibrosing interstitial pneumonia of
unknown cause, primarily occurring in
older adults, limited to the lungs, and
associated with the histopathologic and/or
radiologic pattern of usual interstitial
pneumonia
CLINICAL PRESENTATION
1) Symptoms- are nonspecific and can be shared with
many pulmonary and cardiac diseases
no symptoms
exertional dyspnea
nonproductive cough
Extrapulmonary symptoms
Weight loss
Low-grade fever
Fatigue
Arthralgias
Myalgias
CLINICAL PRESENTATION
2) Signs
Digital clubbing (25-50%)
Bylateral fine basilar inspiratory crackles
Loud P2 component of the second heart
sound, a fixed split S2 (Pulmonary
hypertension )
Holosystolic tricuspid regurgitation murmur
Edema of the legs
LABORATORY TESTS
Routine laboratory tests are nonspecific for the
diagnosis of idiopathic pulmonary fibrosis.
Some tests may be helpful to exclude other
causes of interstitial lung disease:
Antinuclear antibodies or rheumatoid factor
titers: Positive results in about 30% of patients
with IPF, but the titers are generally not high.
The presence of high titers may suggest a
connective tissue disease
C-reactive protein level
Erythrocyte sedimentation rate
Complete blood cell count- polycythemia (rare)
OTHER TESTS
Pulmonary function tests: Nonspecific
findings of a restrictive ventilatory
defect and reduced diffusion capacity
for carbon monoxide (DL CO)
IMAGING TESTS
1) High-resolution computed
tomography (HRCT) scanning
Is
Sensitive, specific, and essential
for the diagnosis of idiopathic
pulmonary fibrosis.
Show patchy, peripheral,
High-resolution computed
tomography (HRCT)
scanning
shows increased
pulmonary attenuation
with distortion of
the pulmonary
architecture.
2) Chest radiography
Show peripheral reticular opacities
(netlike linear and curvilinear
densities) predominantly at the
lung bases, honeycombing (coarse
reticular pattern), and lower lobe
volume loss
Honeycombing
image
CHEST X-RAY
interstitial shadowing
of small (1- to 2-mm),
irregular opacities
OTHER TESTS
3) Transthoracic echocardiography
Detects pulmonary hypertension
4) Bronchoscopy
Absence of lymphocytosis in bronchoalveolar
lavage fluid may be important for the diagnosis
(increased neutrophils [70-90% of patients] and
eosinophils [40-60% of all patients]).
This procedure may be used to exclude
alternative diagnoses.
5)Surgical lung biopsy
(via open lung biopsy or
video-assisted
thoracoscopic surgery
[VATS])
Best sample for
distinguishing usual
interstitial pneumonia
from other idiopathic
interstitial pneumonias.
Three ports are used,
with camera in center
position of triangle.
MANAGEMENT
The optimal medical therapy for the treatment
of idiopathic pulmonary fibrosis has yet to be
identified.
Treatment strategies for idiopathic pulmonary
fibrosis include the assessment and
management of comorbid conditions according
to current practice guidelines, including chronic
obstructive pulmonary disease, obstructive
sleep apnea, gastroesophageal reflux disease,
and coronary artery disease.
Encourage tobacco users to quit and offer
pharmacotherapy as needed.
TREATMENT
Oxygen therapy in patients with
hypoxemia at rest or with exercise (partial
pressure of oxygen [PaO 2] < 55 mmHg or
an oxygen saturation by pulse oximetry
[SpO 2] < 88%). The goal is to maintain an
oxygen saturation of at least 90% at rest,
with sleep, and with exertion.
Vaccinate
patients against influenza and
pneumococcal infection.
Lung transplantation
Pharmacotherapy: