Schaefer-Prokop
Cystic
Lung
Diseases
Fleischner Glossary
Definition
Bulla
round, thin-walled (<1mm), >1cm
accompanied by emphysema
Cyst
round, thin-walled (<2mm)
interface with normal lung
Paraseptal emphysema
no walls , intact interlobular septa,
+ bulla („blebs“ <1cm)
Cystic Lung Disease
Are we dealing with true cysts ?
Are the cysts subpleural
Emphy
sema
LCH DIP
Chr Sarcoi
EAA dosis
UIP
LIP BHD
Are we dealing with true cysts ?
Cavities (e.g., Tb) Mimics
thick-walled (>4mm)
Centrilobular emphysema
no walls, central dot
Cystic bronchiectasis
signet-ring sign, mosaic pattern
Honeycombing
endstage fibrosis
Cystic Bronchiectasis
Silico-Tb
Cysts in the subpleural space ?
Bullae
Paraseptal emphysema
Honeycombing
one or several layers of
subpleural cysts, that share
the wall
UIP
Interobserver Variability
honeycombing
Solitary
incidental, pneumatocele,
bronchogenic cysts
Multiple
Birt-Hogg-Dube with rec pneumothoraces
LAM
LIP with ground glass
PjP
LCH with nodules
BHD
Birt Hogg Dubé Syndrome (BHD)
Frequent pneumothoraces
Up to 90% have lung cysts,
- 2mm-8cm,
- lower zone predominance
- lobulated multiseptated
- thin walled
the remaining lung is normal
LAM
Lymphangioleiomyomatosis (LAM)
HRCT Findings
in young women
LAM
Lymphocytic Interstitial Pneumonia (LIP)
Rarely idiopathic
In association with:
Sjögren’s syndrome
Immune deficiency
syndromes, AIDS
Primary biliary cirrhosis
Multicentric Castlemean’s
disease
LIP in Sjoegren
LIP and Amyloidosis
Langerhans Cell Histiocytosis
Langerhans Cell Histiocytosis
1 year later
Emphy
sema
LCH DIP
Chr Sarcoi
EAA dosis
UIP
LIP BHD
Chronic Exogenic Allergic Alveolitis EAA
Pigeons at home
Lymphocytosis in BAL
Chronic EAA
UIP ??
Sarcoidosis
Sarcoidosis
Fibrobullous Destruction
Emphy
sema
LCH DIP
Chr Sarcoi
EAA dosis
UIP
LIP BHD