Chest Flashcards
how do you differentiate emphysema from cystic lung disease?
Cysts
- centrilobular core structures absent
- thin-walled
Emphysema
- wall - none
- centrilobular core structures
Name different types of cystic lung disease?
- pulmonary Langerhans cell histiocytosis (LCH)
- lymphangioleiomyomatosis (LAM)
- birt-hogg- dube
- lymphocytic interstitial pneumonitis (LIP)
- amyloidosis
- light chain deposition disease
- neurofibromatosis
What is Langerhans cell histiocytosis?
It has a strong association with smoking.
It can be an isolated disease or affect multiple organs.
Typically age 20-40 years
Affects men and women equally
Imaging findings on pulmonary langerhans cell histocytosis?
- nodules (early) - upper lung predominance, irregular and randomly distributed
- cysts (late) - upper lung predominance, thin walls, rounded in shape and increase as the disease progresses.
- upper lung
- bases are spared
- these patients often get a spontaneous pneumothorax
LAM - lymphangioleiomyomatosis.
What is it?
- abnormal smooth muscle proliferation (LAM cells)
- cystic destruction of the lung parenchyma
- the presence of abdominal tumours (angiomyolipomas and lympahngioleiomyomas)
- Women-
cystic lung disease and effusion think LAM (and raised the suggestion of a chylous effusion) + women
LAM imaging features?
- smooth thin-walled cysts
- DIFFUSE - bases are involved
- no nodules
- possible associated pleural effusion (chylous)
- possible associated renal angiomylipomas or retroperitoneal lymphangioleiomyomas
What is Birt-Hogg-Dube Syndrome?
- autosomal dominant
- skin lesions
- cystic lung disease with a lower lung predominance (can have a sub-pleural distribution)
- renal lesions including RCC
What is LIP? (Lymphocytic interstitial pneumonia)
- associated with Sjogrens syndrome
- lower lung predominance
- associated ground glass opacities with a lower lung predominance
- can have associated MALT lymphoma
think thin-walled cyst and ground glass opacities
Describe amyloid cystic lung disease?
- 50% associated with Sjogrens
- cystic lung disease, lower lung predominance and associated pulmonary nodules.
- cysts are often peribronchovascular and subpleural
- can be an association with MALT lymphoma.
Describe pulmonary neurofibromatosis?
- lung disease associated with neurofibromatosis
- 50% have basilar reticular opacities
- 37% have ground glass opacities
25% have cystic lung disease
what do cysts look like in pulmonary neurofibromatosis?
small cysts with thin walls
upper lung predominant
can be seen with other abnormalities such as bullae and ground glass opacities
Distribution and diagnosis of cystic lung disease?
Upper lung > PLCH (smoker)
Diffuse > LAM (women)
What are the common findings associated with patterns of lung disease?
