2. ■ A group of diffuse lung diseases associated with chronic airflow obstruction includes:
chronic obstructive pulmonary disease (COPD),
asthma,
and obliterative bronchiolitis.
3. Chronic obstructive pulmonary disease (copd)
■ The Global Initiative for Chronic Obstructive Pulmonary (Lung) Disease (GOLD) (56) defines COPD in functional
terms, as persistent, usually progressive airflow limitation established spirometrically, as a decrease in the FEV1/FVC
to less than 0.70, that cannot be reversed following bronchodilatortherapy.
■ Two main components of copd are :
emphysema
Chronic bronchitis
4. EMPHYSEMA
• Emphysema is a pathologic diagnosis that is defined as an abnormal, permanent enlargement of the airspaces distal to
the terminal bronchiole, accompanied by destruction of alveolar walls, and without obvious fibrosis.
5. Etiology and Pathogenesis.
■ There is also a causal relationship between HIV infection and
the development of early emphysema.
■ Various genetic disorders may be associated with emphysema
including α1-antitrypsin deficiency, heritable diseases of
connective tissue such as cutix laxa, Marfan syndrome and
familial emphysema.
6. CLASSIFICATION OF EMPHYSEMA
■ The pathologic classification of emphysema is based on the portion of the secondary pulmonary lobule affected:
■ CENTRILOBULAR
■ PANLOBULAR
■ PARASEPTAL
■ IRREGULAR
12. BULLOUS EMPHYSEMA
■ Syndrome of bullous emphysema, or giant bullous emphysema known as “vanishing lung syndrome” or “primary bullous
disease of the lung” –is the presence of large (occupy at least one third of a hemithorax), progressive upper lobe bullae, which
occupy a significant volume of a hemithorax and often asymmetric.
13.
14.
15. CHEST RADIOGRAPH
■ Hyperinflated lung,
Low hemidiaphragm (at or below 7th anterior rib)Flat hemidiaphragm (<1.5 cm distance between line connecting the costo and
cardiophrenic angles and top of midhemidiaphragm)
Retrosternal air space >2.5 cm
“Barrel chest" -enlarged antero-posterior chest diameter
Saber-sheath trachea
Pulmonary vascular pruning and distortion(± pulmonary arterial hypertension)
27. CHRONIC BRONCHITIS
■ It is defined as a productive cough occurring on most days for at least 3 consecutive months and for not less than 2
consecutive years.
■ Other causes of chronic productive cough, including bronchiectasis, tuberculosis, and other chronic infections, must
be excluded before the diagnosis of chronic bronchitis can be made.
29. ■ Bronchial submucosal hyperplasia, smooth muscle hypertrophy, chronic inflammation and the obstruction of small
airways.
■ Airflow obstruction, which is concentrated in the small bronchioles has reversible (mucous plugging, inflammation,
smooth muscle hypertrophy) and irreversible components (fibrosis and stenosis).
30. Radiographic Features
■ CXR:
■ -No abnormality in 21–30% of patients,
■ -Overinflation and oligaemia,
■ -”Tram line "appearance –are parallel lines spaced approx. 3mm apart.
■ -Bronchial wall thickening
■ -Accentuation of linear lung markings.
■ -‘Dirty chest’
■ -Sabre-sheath trachea
35. ASTHMA
■ Asthma is a chronic inflammatory condition involving the airways that causes bronchial hyper-
responsiveness and induces recurrent episodes of wheezing, breathlessness, chest tightness and coughing,
usually associated with widespread but variable airflow obstruction that is often reversible either
spontaneously or after bronchodilator inhalation.
36.
37.
38.
39. Radiographic Features
■ ACUTE SIGNS:
■ CXR:
■ -overinflation and air trapping: flattened diaphragmatic dome
■ -Deepened retrosternal air space
■ -Peribronchial cuffing (inflammation of airway wall)
■ -Bronchial dilatation
■ -Localised areas of hypoattenuation
■
■ CHRONIC CHANGES:
■ CXR:
■ -Normal in 73%,
■ -Central ring shadows - bronchiectasis
■ -Scars (from recurrent infections)
40. ■ CT in asthma has been restricted to the identification of associated conditions, such as ABPA and
detection of condition that may mimic asthma, such as hypersensitivity pneumonitis, obliterative
bronchiolitis, Churg-Strauss syndrome or chronic eosinophilic pneumonia
41.
42.
43.
44. OBLITERATIVE (CONSTRICTIVE) BRONCHIOLITIS
■ Obliterative bronchiolitis is a condition characterized by bronchiolar and peribronchiolar inflammation and fibrosis that
ultimately leads to luminal obliteration affecting membranous and respiratory bronchiolitis
45.
46. ■ The chest radiograph is often normal. In a small number of patients, mild hyperinflation, subtle peripheral
attenuation of the vascular makings, widespread and conspicuous abnormalities in lung attenuation, and
central bronchiectasis may be seen.
47. ■ HRCT (expiration-inspiration images):
■ “Mosaic perfusion" of lobular air trapping (85-100%)
■ Bronchial wall thickening (87%)
■ Bronchiectasis (66-80%)
■ Patchy air trapping on expiratory scans
■ Poorly defined nodular areas of consolidation
■ “Tree-in-bud" appearance
■ Centrilobular ground-glass opacities.
In obstructive lung disease, decreased expiratory flow may be related to loss of lung recoil or small airway obstruction or a combination of both. The most common abnormality correlated with loss of recoil is emphysema. The process that causes the small airway obstruction is inflammatory in nature and is characterised by thickening of all the layers of the bronchiolar walls as well as an accumulation of mucus in the airway lumen (COPD and asthma), and/or an irreversible fibrosis (COPD and obliterative bronchiolitis).
COPD is a complex disorder involving both pulmonary and extrapulmonary components, in which genetic factors interact with environmental antigens, especially tobacco smoke, resulting in an exaggerated inflammatory response that ultimately destroys lung parenchyma, resulting in a loss of elastic recoil (emphysema), and/or remodels the peripheral airways due to mucous gland hyperplasia, bronchoconstriction, and airway narrowing (chronic bronchitis and/or small airway disease)
The acinus is the air-exchanging unit of the lung, and it is located distal to the terminal bronchiole. It includes the respiratory bronchioles, alveolar ducts, alveolar sacs, and alveoli (F
Emphysema is thought to result from the destruction of elastic fibres caused by an imbalance between proteases and protease inhibitors in the lung and from the mechanical stresses of ventilation and coughing. Proteases are normally released in low concentration by phagocytes in the lung. Protease inhibitors, mainly α1-protease inhibitor (α1- antitrypsin), prevent them from causing structural damage to the lung. Imbalance in the protease-antiprotease activity may result from anti- protease deficiency (α1-antitrypsin deficiency), from excess release of protease stimulated by environmental agents, or from the defective repair of protease-induced damage. Tobacco smoke increases the number of pulmonary macrophages and neutrophils, reduces antiprotease activity, and may impair the synthesis of elastin. As emphysema develops, lung destruction progresses, airspaces enlarge and elastic recoil declines, reducing radial traction on bronchial walls and on blood vessels and allowing airways and vessels to collapse.
most common and is characterized by airspace distention in the central portion of the lobule, with sparing of the more distal portions of the lobule. This form of emphysema affects the upper lobes to a greater extent than the lower lobes
distal alveoli spared,
-Severity of destruction varies from lobule to lobule
smokers (in up to 50%), coal workers
Causes:
-Excess protease with smoking (elastase is contained in neutrophils and macrophages found in lung of smokers)
uniform distention of the airspaces throughout the substance of the lobule, from the central respiratory bronchioles to the peripheral alveolar sacs and alveoli. In contrast to centrilobular emphysema, this form has a predilection for the lower lobes
Cause: Autosomal recessive alpha1-antitrypsin deficiency in 10-15% (proteolyticenzymes carried by leukocytes in blood gradually destroy lung unless inactivated by alpha-1 protease inhibitor)
is seen as selective distention of peripheral airspaces adjacent to interlobular septa, with sparing of the centrilobular region. This form of emphysema is most often seen in the immediate subpleural regions of the upper lobes (Fig. 16.15) . Paraseptal emphysema may coalesce to form apical bullae; rupture of these bullae into the pleural space may give rise to spontaneous pneumothoraces.
predominant involvement of alveolar ducts and sacs.
Site: within subpleural lung and adjacent to interlobular septa and vessels
Paracicatricial or irregular emphysema refers to destruction of lung tissue associated with fibrosis that bears no consistent relationship to a given portion of the lobule.It is most often seen in association with old granulomatous inflammation
Is the presence of emphysema associated with large bullae.
-Seen with centrilobular or paraseptal emphysema,
-Young men .
Causes: In smokers, but may also occur in nonsmokers.
Bullae increase progressively in size over time, rarely, spontaneously decrease in size or disappear, as a result of secondary infection or obstruction of the proximal airway.
Spontaneous pneumothorax is common.
Bullous emphysema in a young man.
A)Large bullae are visible in the upper lobes, with displacement of normal lung to the bases.
B)Bullae walls are visible (arrows).
Chest radiograph shows lucency in the lung apices. The right upper lobe is increased in volume with mediastinal shift to the left. Normal lung is compressed at the lung bases.
Hyperinflation is the most important plain radiographic finding and reflects the loss of lung elastic recoil. It is the radiographic equivalent of an abnormally increased total lung capacity
Absent or attenuated peripheral vascular markings are caused by parenchymal destruction and obliteration of peripheral pulmonary arteries traversing emphysematous areas
PA radiograph shows lung height of 29.9cm or more from the dome of the right diaphragm to the tubercle of the first rib and flattening of right hemidiaphragm with highest level of the dome is less than 1.5cm above a perpendicular line drawn between the costophrenic and cardiophrenic angles.
B) Lateral radiograph shows flattening of right hemidiaphragm , increased retrosternal space measuring more than 4.4cm at the level of 3cm below the manubriosternal junction and sternodiaphragmatic angle measures >90 degrees or more.
Axial CT image shows multiple, round areas of low attenuation without visible walls. A centrilobular location can be recognized for several of the focal lucencies (arrows). B, Coronal reformation image shows that the emphysema predominates in the upper lobes.
Emphysematous spaces"(focal round area of air attenuation)> l cm in diameter with central dot or line- centrilobular location (due to centrilobular artery of secondary pulmonary lobule) without definable wall and surrounded by normal lung
-
In advanced stage- pulmonary vascular distortion and pruning with lack of juxtaposition of normal lung
Diffuse simplification of lung architecture with pulmonary septal and vascular distortion and pruning (difficult to detect early),
-Paucity of vessels
-Bullae
Figure 10-9 Panlobular emphysema in a patient with α1-antitrypsin deficiency and a history of cigarette smoking. A, Axial CT image shows a pronounced paucity of vessels in both lower lobes, sometimes referred to as a simplification of lung architecture. “Empty” secondary pulmonary lobules nearly devoid of vessels can be identified (arrow). B, Coronal reformation image shows the lower lobe predominance of panlobular emphysema and less severe centrilobular emphysema in the upper lobes.
10 Paraseptal emphysema. A, Axial CT image shows many areas of emphysema localized to the subpleural regions bilaterally and a bulla is noted on the right medially (arrow). B, Coronal reformation image shows upper lobe distribution of emphysema
Paraseptal and centrilobular emphysema in smoker.
A)HRCT at the lung apices shows extensive emphysema with bullae. The subpleural bullae represents paraseptal emphysema.
B) At a lower level, more discrete areas of paraseptal emphysema and subpleural bullae are visible(arrows).
Emphysema within the central upper lobes is centrilobular.
At a level below that seen in previous image (B)
The areas of emphysema appears smaller and occurs in a single layer
HRCT shows subpleural airspaces (arrows) and associated centrilobular emphysema.
Paracicatricial or irregular emphysema (Fig. 10-11) is focal emphysema that is usually found adjacent to parenchymal scars, in diffuse pulmonary fibrosis, and in the pneumoconioses, particularly when progressive, massive fibrosis is present. It is usually recognized on CT when the associated fibrosis is identified.
Focal emphysema (arrow) surrounds an old tuberculous cavity with an intracavitary fungus ball.
The first is honeycombing, which occurs in pulmonary fibrosis and is characterized by areas of subpleural cystic lesions that somewhat mimic the appearance of paraseptal emphysema. However, honeycomb cysts are usually smaller; they occur in several layers along the pleural surface, are localized in the lung bases, and are associated with other findings of fibrosis. Paraseptal emphysema is often associated with bullae, and the areas of emphysema are larger and occur in a single layer. They predominate in the upper lobes without evidence of fibrosis
pneumatocele is a thin-walled, gas-filled space within the lung that usually is associated with acute pneumonia or more chronic infections, such as Pneumocystis jiroveci (formerly P. carinii) pneumonia. They are usually transient and can be identical to bullae on HRCT. However, the association with current or previous infection should suggest the diagnosis.
The third entity is cystic lung disease. Multiple, thin-walled lung cysts can be seen in a variety of disorders, particularly the infiltrative lung diseases, such as lymphangioleiomyomatosis and Langerhans cell histiocytosis. These cysts usually can be differentiated from centrilobular emphysema because the walls are more distinct and lung cysts appear larger. When the lucency can be clearly identified as within the center of the pulmonary lobule, it is diagnostic of centrilobular emphysema. Cystic bronchiectasis can be readily differentiated from lung cysts and emphysema based on connectivity with the proximal airways and a constant relationship with its accompanying pulmonary artery.
Bronchiolitis obliterans, a disease of the small airways, can result in increased lung volume and oligemia that is similar to that of panlobular emphysema. However, it usually has a patchy distribution, which is an important distinguishing feature. Some dilation of the central airways with mild bronchiectasis is more common in patients with bronchiolitis obliterans than in patients with panlobular emphysema.
Interstitial Lung Abnormalities in a Chronic Obstructive Pulmonary Disease Patient. (A) Axial computed tomography images in upper and lower parts of the lungs. (B) Coronal and right sagittal reformations. Paraseptal and confluent centrilobular emphysema are seen in the upper lobes. Posterior subpleural paraseptal emphysema is seen in the lower lobes. Presence of reticulation in the peripheral parts of the lower lobes surrounding enlarged airspaces of emphysema in subpleural parenchyma mimicking honeycombing of usual interstitial pneumonitis (UIP).
Most patients with chronic bronchitis are smokers, and they often have emphysema.
- narrowing of the coronal diameter (tracheal index (the ratio of the coronal to the sagittal diameter 1 cm above the aortic arch) is less than 0.67).
-increased lung markings and loss in clarity of the lung vessels,
– tubular ring shadow
Frontal chest radiograph in a patient with a history of chronic bronchitis and chronic obstructive pulmonary disease shows hyperinflation with increased parenchymal markings. B, C: Sagittal maximum intensity projection CT scans through the right (B) and left (C) lungs show upper lobe predominant centrilobular tree-in-bud opacities (arrowheads) and mosaic attenuation (asterisks in C), the latter likely due to air trappin
HRCT in a patient with symptoms of chronic bronchitis.
A) Inspiratory scan shows airway wall thickening (arrows) in the lower lobe, without evidence of bronchiectasis.
B) Expiratory scan shows air trapping (arrows) in this region.
Age: middle age
PATHOGENESIS: autoimmune phenomenon caused by viral respiratory infection , exercise, and pharmaceuticals.
No environmental antigen
B. EXTRINSIC ASTHMA= ATOPIC ASTHMA
PATHOGENESIS:
-Secondary to antigens producing an immediate hypersensitivity response (type I),
-Sensitizes mast cells to release histamine,
-Increased vascular permeability and edema ,
-Small muscle contraction leads to bronchial constriction causing airway obstruction
Chest radiograph may depict complications including consolidation, atelectasis, mucoïd impaction, pneumothorax or pneumomediastinum. Consolidation is commonly infective but, in some cases, it is due to eosinophilic consolidation, probably associated with allergic aspergillosis.
Mucoid impaction with allergic bronchopulmonary aspergillosis (ABPA) in an asthmatic patient. A, Branching, V-shaped, mucus-filled, dilated bronchi are identified in the right lung anteriorly. B, The mucous plugs in the lower lobes are oval or round (arrows). Bronchi are slightly dilated with wall thickening (arrows).
Axial computed tomography images at the levels of the middle (A) and lower (B) parts of the lungs. Diffuse bronchial wall thickening with mucoid impactions in the segmental and subsegmental bronchi in the basilar segments of the right lower lobe. Patchy areas of hypoattenuation in the anterior, lateral and posterobasal segments of the right lower lobe and the posterior segment of the left lower lobe r
The pattern of obliterative bronchiolitis is characterised by the develop- ment of an irreversible circumferential submucosal fibrosis, resulting in bronchiolar narrowing or obliteration of bronchioles in the absence of intraluminal granulation tissue polyps or surrounding parenchymal inflammation
patchy areas of decreased lung attenuation alternating with areas of normal attenuation:
(due to collateral air drift into postobstructive alveoli),-4
- centrilobular branching structures and nodules caused by peribronchiolar thickening and bronchiolectasis with secretions,
Mosaic perfusion and bronchiectasis with bronchiolitisobliterans.
A) A patient with chronic lung transplant rejection showsbronchiectasis and mosaic perfusion on HRCT.