Difference between revisions of "Idiopathic interstitial pneumonias"
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− | + | *Rare | |
+ | *Reaction to lung injury | ||
+ | *Variable inflammation and fibrosis | ||
+ | *Variable response to treatment | ||
− | '''[[Idiopathic pulmonary fibrosis]] (IPF)''' | + | Common disorders such as [[sarcoidosis]], vasculitis and connective tissue disorders display an identicle morphologic pattern. Bleomycin drug toxicity can also give a similar appearance. |
+ | |||
+ | |||
+ | '''[[Idiopathic pulmonary fibrosis]] (IPF)''' | ||
+ | *Usual interstital pneumonia (UIP) is the histologic pattern | ||
*Basal and peripheral reticular opacities | *Basal and peripheral reticular opacities | ||
− | *'''Honeycombing''' | + | *'''Honeycombing''', subpleural cysts |
− | *Traction bronchiectasis | + | *Traction bronchiectasis due to surrounding fibrosis |
*Apicobasal gradient - gets worse from apex to base | *Apicobasal gradient - gets worse from apex to base | ||
*Limited ground glass opacities | *Limited ground glass opacities | ||
*Heterogeneous - diseased lung alternating with healthy lung | *Heterogeneous - diseased lung alternating with healthy lung | ||
− | *Exclusion of known causes (toxins, environmental exposure, etc) | + | *Exclusion of known causes (toxins, environmental exposure, collagen vascular disease, etc) |
− | * | + | *Reduced lung volume on x-ray |
+ | *Mean survival 3 years | ||
Revision as of 17:49, 1 June 2012
- Rare
- Reaction to lung injury
- Variable inflammation and fibrosis
- Variable response to treatment
Common disorders such as sarcoidosis, vasculitis and connective tissue disorders display an identicle morphologic pattern. Bleomycin drug toxicity can also give a similar appearance.
Idiopathic pulmonary fibrosis (IPF)
- Usual interstital pneumonia (UIP) is the histologic pattern
- Basal and peripheral reticular opacities
- Honeycombing, subpleural cysts
- Traction bronchiectasis due to surrounding fibrosis
- Apicobasal gradient - gets worse from apex to base
- Limited ground glass opacities
- Heterogeneous - diseased lung alternating with healthy lung
- Exclusion of known causes (toxins, environmental exposure, collagen vascular disease, etc)
- Reduced lung volume on x-ray
- Mean survival 3 years
Nonspecific intersitial pneumonia (NSIP)
- Basal ground glass opacitis predominate over reticulat opacities
- UIP without honeycombing
- Subpleural sparing
- Potentially reversible with immunosupression
Cryptogenic organizing pneumonia (COP)
- Patchy peripheral subpleural or peribronchovascular consolidation
- Atoll sign
- Idiopathic inflammation of the bronchioles and surrounding tissue causing granulation tissue and "non-infectious pneumonia"
- Responds to steroids not antibiotics
- a/w many systemic diseases (lupus, c-anca, GVHD, etc)
- No pleural effusion or lymphadenopathy
Respiratory Bronchiolitis associated Interstitial Lung Disease (RB ILD)
- Smoking related disease
- Centrilobular nodules
- Ground glass opacities
- Upper lobe predominant
Desquamative Intersitial Pneumonia (DIP)
- Smoking related disease
- Centrilobular nodules
- Ground glass opacities
Lymphoid Intersitial Pneumonia (LIP)
- Groung glass opacities
- Cystic lesions
Acute Interstial Pneumonia (AIP)
- Diffuse lung consolidation
- Groung glass opacities
- Progress to fibrosis after acute phase