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Idiopathic pulmonary fibrosis (complications (pulmonary HTN, acute…
Idiopathic pulmonary fibrosis
definition
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
aetiology
?
exposure to an inciting agent (eg, smoke, environmental pollutants, environmental dust, viral infections, gastroesophageal reflux disease, chronic aspiration) in a susceptible host may lead to the initial alveolar epithelial damage.
pathophysiology
?
Epithelial-fibroblastic disease, in which unknown endogenous or environmental stimuli disrupt the homeostasis of alveolar epithelial cells, resulting in diffuse epithelial cell activation and aberrant epithelial cell repair.
incidence
M>F
over 50y
10.7 per 100,000 person years
prognosis
poor
mean surivival 2-5y
clinical
symptoms
nonspecific
exertional dyspnoea
nonproductive cough
associated systemic symptoms
weight loss
fatigue
arthalgia
low-grade fever
signs
digital clubbing
fine bibasal crackles
pulmonary HTN
complications
pulmonary HTN
acute exacerbation of pulmonary fibrosis
respiratory infection
acute coronary syndrome
thromboembolic disease
lung cancer
diagnosis
CT
ancillary
CXR
peripheral reticular opacities
honey combing
pulmonary function tests
treatment
medical
oxygen therapy
smoking cessation
vaccination
surgical
lung transplant