Cystic Lung Lesions

By

Gamal Rabie Agmy , MD , FCCP Professor of Chest Diseases ,Assiut University

DECREASED LUNG ATTENUATION

Lung Cysts

Pulmonary fibrosis (Honeycombing)

Lymphangiomyomatosis

Langerhanscell histiocytosis

Lymphocytic Interstitial Pneumonia (LIP)

Differential Diagnosis

Rough Reticular Fine Reticular

Traction

Bronchiectasis

and

Interface

sign

Honey

combing

UIP UIP or NSIP

Usual Interstitial Pneumonia UIP

HRCT Findings

Reticular opacities, thickened intra- and

interlobular septa

Irregular interfaces

Honey combing and parenchymal distorsion

Ground glass opacities (never prominent)

Basal and subpleural predominance

Basal and subpleural distribution

UIP

The Many ‘HRCT Faces’ of NSIP

Honeycombing not a

prominent feature !!!!

Lymphangioleiomyomatosis (LAM)

HRCT Morphology

Thin-walled cysts (2mm - 5cm)

Uniform in size / rarely confluent

Homogeneous distribution

Chylous pleural effusion

Lymphadenopathy

in young women

Lymphangioleiomyomatosis (LAM)

Tuberous Sclerosis (young man)

Langerhans Cell Histiocytosis

Langerhans Cell Histiocytosis

HRCT Findings

Small peribronchiolar nodules (1-5mm)

Thin-walled cysts (< 1cm),

Bizarre and confluent

Ground glass opacities

Late signs: irreversible / parenchymal fibrosis Honey comb lung, septal thickening, bronchiectasis

1 year later

Peribronchiolar Nodules Cavitating nodules and cysts

Langerhans Cell Histiocytosis

Langerhans Cell Histiocytosis

Langerhans Cell Histiozytosis

Key Features

Upper lobe predominance

Combination of cysts and noduli

Characteristic stages

Increased Lung volume

Sparing of costophrenic angle

S

M

O

K

I

N

G

Langerhans Cell Histiocytosis

Langerhans Cell Histiocytosis

Differential Diagnosis

Only small nodules Sarkoidosis, Silikosis

Only cysts idiopathic Fibrosis

LAM

Destruktive emphysema

A professional diver.............

.......after cessation of smoking

Benign lymphoproliferative disorder Diffuse interstitial infiltration of

mononuclear cells

Not limited to the air ways as

in follicular Bronchiolitis

LIP

= Lymphocytic Interstitial Pneumonia

Sjögren: LIP

LIP

= Lymphocytic Interstitial Pneumonia

Rarely idiopathic

In association with: Sjögren‟s syndrome

Immune deficiency syndromes, AIDS

Primary biliary cirrhosis

Multicentric Castlemean‟s disease

Sjoegren disease

Dry eye and dry mouth

Fibrosis, bronchitis and bronchiolitis

LIP

Overlap

Sarcoid, DM/PM, MXCT

SLE, RA (pleural effusion)

Up to 40 x increased risk for lymphoma (mediastinal

adenopathy) and

2 x times increased risk for neoplasma

Summary..................................Quiz

Young woman Dry mouth Smoker

LAM LIP Histiocytosis

Emphysema Fibrosis (UIP)

Wegener„s disease

Rheumatoid Arthritis

Emphysema

histopathological definition

…..permanent abnormal enlargement of airspaces

distal to the bronchioles terminales and

…...destruction of the walls of the involved

airspaces

Centrilobular Emphysema

Panlobular Emphysema

CLE and PLE in one Patient

Fibrosis and Emphysema

What is Your Diagnosis ?

Cystic Changes and Decreased Density

Quiz

LAM Emphysema Fibrosis

LCH Emphysema

Fibrosis Emphysema

Emphysema

Emphysema typically presents as

areas of low attenuation without

visible walls as a result of

parenchymal destruction.

EMPHYSEMA

Permanent, abnormal enlargement of air

spaces distal to the terminal bronchiole and

accompanied by the destruction of the walls

of the involved air spaces.

43

Centrilobular emphysema

Most common type

Irreversible destruction of alveolar walls

in the centrilobular portion of the lobule

Upper lobe predominance and uneven

distribution

Strongly associated with smoking.

Centrilobular (proximal or

centriacinar) emphysema Found most commonly in the upper lobes

Manifests as multiple small areas of low attenuation without a

perceptible wall, producing a punched-out appearance.

Often the centrilobular artery is visible within the

centre of these lucencies.

45

Centrilobular emphysema due to smoking. The periphery of the

lung is spared (blue arrows). Centrilobular artery (yellow arrows)

is seen in the center of the hypodense area.

Panlobular emphysema

Affects the whole secondary lobule

Lower lobe predominance

In alpha-1-antitrypsin deficiency, but

also seen in smokers with advanced

emphysema

PANLOBULAR EMPHYSEMA

Affects the entire secondary pulmonary lobule

and is more pronounced in the lower zones

Complete destruction of the entire pulmonary

lobule.

Results in an overall decrease in lung

attenuation and a reduction in size of

pulmonary vessels

48

PANLOBULAR EMPHYSEMA

49

Panlobular emphysema

Paraseptal (distal acinar)

emphysema

Affects the peripheral parts of the secondary

pulmonary lobule

Produces subpleural lucencies.

51

Paraseptal emphysema

Cystic lung disease

Lung cysts are defined as radiolucent areas with a

wall thickness of less than 4mm.

Langerhans cell histiocytosis

Lymphangiomyomatosis complicated by pneumothorax

Bronchiectasis

Bronchiectasis is defined as localized bronchial

dilatation. (signet-ring sign)

bronchial wall thickening

lack of normal tapering with visibility of airways in

the peripheral lung

mucus retention in the broncial lumen

associated atelectasis and sometimes air trapping

ABPA: glove-finger shadow due to mucoid impaction in central

bronchiectasis in a patient with asthma.

Signet-Ring Sign

A signet-ring sign represents an axial cut of a dilated bronchus

(ring) with its accompanying small artery (signet).

Tram Tracks

Bronchial dilation with lack of tapering .

HONEYCOMBING

Defined as - small cystic spaces with irregularly

thickened walls composed of fibrous tissue.

Predominate in the peripheral and subpleural

lung regions

Subpleural honeycomb cysts typically occur in

several contiguous layers. D/D- paraseptal

emphysema in which subpleural cysts usually

occur in a single layer.

Indicates the presence of “END stage” disease

regardless of the cause.

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Honeycombing

Honeycombing is defined by the presence of small cystic

spaces with irregularly thickened walls composed of fibrous

tissue.

Causes

Lower lobe predominance :

1. UIP or interstitial fibrosis

2. Connective tissue disorders

3. Hypersensitivity pneumonitis

4. Asbestosis

5. NSIP (rare)

Upper lobe predominance :

1. End stage sarcodosis

2. Radiation

3. Hypersensitivity Pneumonitis

4. End stage ARDS

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Honeycombing

HRCT showing

subpleural

broncheolectasis

Honeycombing and traction bronchiectasis in UIP.

Typical UIP with honeycombing and traction

bronchiectasis in a patient with idiopathic

pulmonary fibrosis (IPF)

Distribution within the lung

Additional findings