Idiopathic Pulmonary Fibrosis Flashcards
idiopathic pulmonary fibrosis
unknown cause
involves lung only
chronic, progressive, irreversible, and usually lethal
pathogenesis
repeated stimulus leads to sequential lung injury and inflammation
the result is aberrant wound healing and fibrosis
inflammation is mediated by genetic factors and the Th1-Th2 balance
risk factors for IPF
smoking
farming and livestock
sand, stone or silica
wood dust
metal dust
clinical history of iPF
chronic cough for months - dry, nonproductive, unresponsive to medications
chronic dyspnea for months - progressive, starts as decreased effort tolerance and progresses to dyspnea at rest
phyiscal examination findings in IPF
clubbing
bilateral basal inspiratory crackles
absences of clues to suggest alternative etiology such as connective tissue disease
HRCT findings for IPF
subplerual, basal predominance
reticular abnormality
honeycominb with or without traction bronchiectasis
absence of features that are inconsistent with a UIP pattern
subacute hypersensitivity pneumonitis
pneumocystis pneumonia
alveolar proteinosis
sarcoidosis
histiocytosis
IPF
definite UIP pattern
evidence of fibrosis/architectural distortion with or without honeycombing in a predominantly subpleural/paraseptal location
fibrosis in a patchy distribution
fibroblast foci
absence of features suggesting not a UIP pattern
possible treatment of IPF
pirfenadone is theonly drug in trials that has been shown to have modest effect
non-pharmocologic therapies for IPF
oxygen supplementation for patients with hypoxia
pulmonary rehabilitation and structured exercise program
palliative care and symptom relief
lung transplantation
