Diffuse Lung disease Flashcards
1
Q
what are clinically present in interstitial lung disease
A
restrictive lung disease and hypoxemia on pulmonary function tests
2
Q
Scaffolding of the lung, providing support for the airways, gas-exchanging units and vascular structures
A
Pulmonary interstititum
3
Q
The central interstitial compartment extending from the mediastinum peripherally and enveloping the bronchovascular bundles is termed the
A
Axial interstitium
4
Q
Axial interstitium is contiguous with the interstitium surrounding the small centrilobular arterieand bronchiole within the secondary pulmonary lobule, where it is called
A
Centrilobular intersititium
5
Q
The most peripheral component of the interstitium is the
A
Subpleural or peripheral interstitium
6
Q
Invaginations of the subpleural interstitium into the lung parenchyma from the borders of the secondary pulmonary lobules and represent the
A
Interlobular septa
7
Q
fine network of connective tissue fibers that support the alveolar spaces
A
intralobular, parenchymal or alveolar interstitium
8
Q
defined as that subsegment of lung supplied by three to five terminal bronchioles and separated from adjacent secondary lobules by intervening connective tissue (interlobular septa)
A
secondary pulmonary lobule
9
Q
each terminal bronchiole subdivide into
A
respiratory bronchioles, alveolar ducts, alveolar sacs and alveoli
10
Q
unit of lung subtended from a single terminal bronchiole is called a
A
pulmonary acinus
11
Q
located in the center of secondary lobule
A
centrilobular artery, preterminal bronchiole
12
Q
present at the margins of lobules within the interlobular septa, found within the contiguous subpleural interstitium
A
lymphatic and connective tissue
13
Q
secondary pulmonary lobule is typically of what shape
A
polyhedral
14
Q
normally 0.1 mm thick and can be seen in lung periphery, particularly along the superior and inferior pleural surfaces
A
interlobular septa
15
Q
are V- or Y- shaped structures on thin-section CT seen within 5 to 10 mm of pleural surface
A
centrilobular arteries
16
Q
normal airways are visible only to within ___cm of the pleura
A
3cm
17
Q
has a diameter of 1mm and a wall thickness of 0.15 mm, not normally visible on thin-section CT
A
centrilobular brochiole
18
Q
occassionally seen as linear or dot-like structures within 1-2 cm of the pleura and when visible, may indicate the locations of interlobular septa
A
pulmonary veins
19
Q
normally not visible on thin section CT
A
peribronchovascular, centrilobular, and intralobular interstitial compartments
20
Q
interlobular lines on thin-section CT are equivalent of _____ lines seen in the inferolateral portions of the lungs on frontal radiographs
A
Kerley B
21
Q
within the central regions of the lung, long (2-6 cm) linear opacities representing obliquely oriented connective tissue septa are the equivalent of ______ lines
A
Kerley A
22
Q
thickened intralobular lines are usually from
A
fibrosis, UIP, IPF
23
Q
seen within the central portion of pulmonary lobule radiating outward the thickened lobular borders to produce a “spoke-and-wheel” or “spider web” appearance
A
intralobular septa
24
Q
nodular fissural thickening are usually seen in
A
sarcoidosis and lympangitic carcinomatosis
25
can either result in smooth or irregular peribronchovascular thickening
lymphangitic carcinomatosis
26
centrilobular abnormalities are usually seen in
subacute hypersensitive pneumonitis, cryptogenic organizing pneumonia, respiratory bronchiolitis-associated interstitial lung disease
27
Probably represent an early phase of lung fibrosis. 5-10cm long curvilinear opacities found within 1cm of the pleura and parallel chest wall
Subpleural lines
28
Small 6-10mm cystic spaces with 1-3mm walls, most often present in the posterior subpleural regions of the lower lobes, and result from end stage pulmonary fibrosis from a variety of etiologies
Honeycomb cysts
29
Nontapering linear opacities 2-5 cm in length, that extend from the lung to contact pleural surface
Parenchymal bands
30
Common manifestation of late stages of Langerhans cell histiocytosis, also known as eosinophilic granulomatosis and lymphangioleiomyomatosis. Slightly larger in diameter and have thinner walls than honeycomb cyst
Thin-walled cysts
31
Area of increased attenuation within which the normal parenchymal structures are visible
Ground glass opacity
32
1-3 mm sharply marginated round opacities seen on thin section CT which represent conglomerates of granulomas or tumor cells within the interstitium
Micronodules
33
Most often produced by thickening of the alveolar septa, with or without the lining of alveolar spaces, by inflammatory exudate or fluid
Ground glass opacities
34
Presence of these opacities is important because it often implies an active inflammatory process of edema that is reversivle and warrants aggressive treatment
Ground glass opacities
35
Ground glass abnormality associated with a predominant pattern of honeycombing indicates
Microscopic pulmonary fibrosis
36
Finding commonly associated with architectural distortion is
Traction bronchiectasis
37
Refers to increased lung density that obscures underlying blood vessels; air bronchograms are commonly present
Consolidation
38
Results from diffuse inflammatory processes that primarily affect the axial and parenchymal interstitium of the lung
Chronic interstitial disease
39
Chronic interstitial pulmonary edema are commonly seen in patients with
Long standing mitral stenosis, or LV failure
40
Redistribution of blood flow to the upper lobes is a manifestation of
Pulmonary venous hypertension
41
Redistribution of blood flow to the upper lobes, prominence of fissures caused by subpleural edema and fibrosis are concomitant findings in pulmonary venous hypertension. Presence of honeycombing is not a feature of chronic PVH, however if present in a patient with cardiac disease, another cause of pulmonary fibrosis such as ____ may be considered
Amiodarone lung toxicity
42
Pulmonary involvement in RA is more common in what gender
Male
43
True or false: pleuropulmonary manifestations of RA typically follow the onser of joint disease and tend to be seen in patients with high serum RA factor titers and eosinophilia
True
44
Most common radiographic manifestation of RA lung involvement
Interstitial pneumonitis and fibrosis
45
Most common thoracic manifestation of rheumatoid disease and is found in 20% of patients
Pleuritis
46
Pleural effusions in RA are transudative or exudative
Exudative, with low glucose concentration
47
May develop from diffuse interstitial fibrosis secondary to rheumatoid disease
Enlargement of central pulmonary arteries and right heart dilatation
48
Chest wall abnormalities that may be seen with RA
Tapered erosion of distal clavicles, rotator cuff atrophy with high-riding humeral head, bilateral symmetric glenohumeral joint space narrowing, with or without superimposed degenerative joint disease and superior rib notching or erosion
49
Thorax is commonly affected and may be the initial site of involvement in SLE. The thoracic disease is often limited to the
Pleura and pericardium
50
In SLE, pleura and pericardium exhibits _____ which produces painful exudative pleural and pericardial effusions
Fibrinous serositis
51
Results in diffuse pleural thickening and is present in the majority of patients with long standing SLE
Pleural fibrosis
52
Pleural effusions in SLE typically improves after what tx
Corticosteroid therapy
53
Characterized by rapid onset of fever, dyspnea and hypoxemia, which may require ventilation in patients with SLE
Acute lupus pneumonitis
54
Produces inflammation and fibrosis of the skin, esophagus, msk, heart, lungs and kidneys in young and middle-aged women
Scleroderma
55
True or false: in scleroderma, lungs are involved pathologically in nearly 90% of patients, altho only 25% have respiratory symptoms or radiographic evidence of pulmonary involvement
True
56
Can be seen in up to 50% of patients with scleroderma and may be seen in the absence of interstitial fibrosis
Pulmonary arterial hypertension with enlarged central pulmonary arteries
57
Pulmonary arterial hypertension in patients with scleroderma results from
Thickening and obliteration of small muscular pulmonary arteries and arterioles
58
True or false, pleural effusions are significantly less common in scleroderma than in rheumatoid disease or SLE
True
59
Common lung ca that may be present with scleroderma
Bronchoalveolar cell carcinoma
60
Involve autoimmune inflammation and destruction of skeletal muscle, producing proximal muscle pain and weakness and occassionally associated with a skin radh
Dermatomyositis and polymyositis
61
Lung ca that is mostly seen in patietns with dermatomyositis or polymyositis
Bronchogenic carcinoma
62
Autoimmune disorder of middle-aged women is characterized by sicca syndrome which results from lymphocytic infiltration of the lacrimal, salivary and mucous glands, respectively
Sjogren syndrome
63
Components of sicca syndrome
Dry eyes (keratoconjunctivitis sicca), dry mouth (xerostamia), dry nose (xerorhinia)
64
Patients with Sjogren syndrome are at increased risk for developing
Lymphocytic interstitial pneumonitis and non-hodgkin pulmonary lymphoma
65
Possible Lung findings in ankylosing spondylitis
Upper lobe pulmonary fibrosis, bullae and cavities which are prone to mycetoma formation with aspergillus
66
Most common form of IIP
Usual IP
67
Distinguishing histologic feature of UIP
Different stages of the disease are seen simultaneously within different portions of the lung (temporal heterogeneity)
68
Pulmonary function tests in UIP presents as
Restrictive disease, and a decreased diffusing capacity of the lungs for carbon monoxide
69
Age of onset and gender predilection of UIP
5th to 7th decades and male predominance
70
Distribution of UIP
Basal predominant, subpleural predominant
71
Most common histologic type of lung cancer in IPF
Adenocarcinoma
72
Many cases of NSIP are seen associated with
Collagen vascular disease or as drug reactions
73
Distribution of ground glass opacities and consolidation in NSIP
Peripheral and lower zone distribution
74
Honeycombing is seen frequently in UIP or NSIP?
UIP
75
Distribution of ggo, small centrilobular nodules in respiratory bronchiolitis associated interstitial pneumonia
Upper lobe
76
Usual concomitant finding in respiratory bronchiolitis-associated interstitial lung disease
Emphysema
77
Interstitial pneumonia characterized by accumulation of macrophages within alveolar spaces
Desquamative interstitial pneumonia
78
IP usually seen in smokers
DIP
79
Typical radiographic findings in DIP
Basilar reticular opacities with normal or minimally diminished lung volumes, mostly often within the peripheral aspects of the bases
80
Characterized by the widespread deposition of granulation tissue (fibroblasts,collagen and capillaries) within peribronchiolar airspaces and bronchioles
Organizing pneumonia or COP
81
Viral infections such as influenza, adenovirus and measles, toxic fume inhalation ( sulfur dioxide, chlorine); collagen vascular disease (RA and SLE); organ transplantation, drug reactions and chronic aspiration shows what type of pneumonia
OP or COP
82
Characteristic histologic changes in COP
Absence of parenchymal distortion and fibrosis
83
Also known as Hamman-Rich syndrome, which is an acute, aggressive form of idiopathic interstitial pneumonitis and fibrosis. Presents with a brief history of cough, fever, and dyspnea that progresses rapidly to severe hypoxemia and respiratory failure requiring mech vent
Acute interstitial pneumonia
84
Rare, potentially fatal disease characterized by elastic tissue-rich fibrosis involving the pleura and adjacent lung parenchyma
Pleuroparenchymal fibroelastosis
85
Most severely involved lobe in pleuroparenchymal fibroelastosis
Upper lobes
86
Thoracic manifestations of NF1
Kyphoscoliosis, scalloping of posterior aspect of vertebral bodies caused by dural ectasia, ribbon rib and rib notching
87
Mediastinal masses in patients with NF1 include
Neurofibromas, lateral thoracic meningoceles, extra-adrenal pheochromocytomas
88
Classic triad of tuberous sclerosis
Seizures, mental retardation and adenoma sebaceum
89
Lymphangioleiomyomatosis is an uncommon condition that is seen exclusively in
Women
90
Rare condition that typically affectd woman of childbearing age who presents with progressive dyspnea or a spontaneous pneumothorax. Hemoptysis may also be seen in some patients, presumambly related to pulmonary venous obstruction by smooth muscle proliferation
Lymphangioleiomyomatosis
91
Drug that may slow the progression of LAM
Antiprogesterone agents such as tamoxifen
92
Type of alveolar septal amyloidosis in which there is no associated chronic disease or in which there is an underlying plasma cell disorder
Primary
93
Type of alveolar septal amyloidosis where there is an underlying chronic abnormality such as TB
Secondary
94
Type of amyloidosis that is usually localized to nervous tissue
Familial
95
Three major patterns of amyloid deposition within the lungs and airways
Tracheobronchial, nodular parenchymal, diffuse parenchymal (alveolar septal)
96
Amyloid is deposited in the parenchymal interstitium and within the media of small blood vessels
Alveolar septal amyloidosis
97
True or false. There is no effective treatment in alveolar septal amyloidosis
True
98
Lung biopsy findings in amyloidosia
Amorphous eosinophilic material thickening the alveolar septa that appears apple green in color when stained with congo redo
99
Used to describe the nonneoplastic reaction of the lungs to inhaled inorganic dust particles
Pneumoconiosis
100
2 types of pathologic reaction in pneumoconiosis
Fibrosis and aggregation of particle-laden macrophages
101
What type of asbestos have a greater fibrogenic and carcinoenic potential
Amphiboles
102
Most common disease associated with asbestosis
Pleural disease, as parietal pleural plaques
103
Pulmonary parenchymal manifestations of asbestosis
Rounded atelectasis, interstitial fibrosis, and bronchogenic carcinoma
104
True or false, development of asbestosis depends on both the length and severity of exposure, and clinical manifestations are usually not apparent for 20 to 40 years following initial exposure
True
105
Most common ct finding in asbestos-exposed individuals
Interlobular septal thickening
106
2 histopathologic reaction to silicosis
Silicotic nodules and silicoproteinosis
107
Silicotic nodules measures ___ and are made up of dense concentric lamellae of collagen
1-10 mm
108
Silicotic nodules are typically most numerous in what regions
Upper lobes and parahilar regions
109
Exposure of how many years before radiographic changes of fibrotic silicosis develops
10-20 years
110
Hilar nodes in silicosis often demonstrate
Eggshell calcification
111
Patients with acute silicoproteinosis have an increased susceptibility to
TB and nocardia
112
2 characteristic pathologic finding in CWP
Coal dust macule and PMF
113
Round or stellate nodules ranging in size from 1-5mm seen in cwp
Coal dust macule
114
Defined as nodular or mass-like lesions exceeding 2-3 cm in diameter that are composed of irregular fibrosis and pigment seen in cwp
PMF
115
PMF in cwp are often seen in what lung segments
Posterior segments of upper lobes and superior segments of lower lobes
116
Also known as rheumatoid pneumoconiosis seen in coal workers with RA, characterized radiographically by nodular opacities 0.5 to 5 cm in diameter that develop rapidly and tend to appear in crops. Nodules are more sharply defined and seen more peripherally than the masses of PMF
Caplan syndrome
117
Pneumoconiosis that produces a reaction that mimics sarcoidosis
Chronic berylliosis
118
Extrinsic allergic alveolitis is an immunologic pulmonary disorder associated with the inhalation of one of the antigenic organic dusts
Hypersensitivity pneumonitis
119
Exposure to moldy hay produce
Farmer’s lung
120
Exposure to water reservoirs contaminated by thermophilic bacteria
Humidifier lung
121
Exposure to avian proteins in feathers and excreta produce
Bird fancier’s lung
122
What type of hypersensitivity reaction is produced in hypersensitivity pneumonitis
Type 3 (immune complex) reaction
123
Radiographic features of early hypersensitivity pneumonitis
Fine nodular or ground glass opacities most often seen in lower lobes
124
Chronic changes in hypersensitivity pneumonitis
Diffuse coarse reticular or reticulonodular opacities in mid lung and upper lung zones, honeycomb pattern with loss of lung volume
125
This disease should be considered when repeated episodes of rapidly changing ground glass or airspace opacification are seen in a patient with underlying coarse interstitial lung disease
Hypersensitivity pneumonitis
126
Cylindrical bronchiectasis in upper and lower lobes appears as multiple dilated thick-walled circular lucencies, with the adjoining artery giving each dilated bronchus the appearanxe of a
Signet ring
127
In the mid-lung, where the bronchi course horizontally, the appearance of bronchiectasis is that of parallel linear opacities called
Tram tracks
128
Detection of varicose bronchiectasis along with the classically described finger-in-glove appearance of mucoid impaction in an asthmatic patient should suggest the diagnosis of
Allergic bronchopulmonary aspergillosis
129
A vicous cycle of recurrent infection in cystic fibrosis is most often caused by what organisms which eventually causes severe bronchiectasis
Pseudomonas aeruginosa or staph aureus
130
True or false: distal atelectasis and obstructive pneumonitis are common findings in cystic fibrosis
True
131
Disorder in which the epithelial cilial motion is abnormal and ineffective
Primary ciliary dyskinesia aka dysmotile cilia syndrome
132
May result in rhinitis, sinusitis,bronchiectasis, dysmotile spermatozoa and sterility, situs inversus, and dextrocardia
Primary ciliary dyskinesia
133
what phase of hypersensitivity pneumonitis: airspace opacities
acute
134
what phase of hypersensitivity pneumonitis: patchy areas of ggo and poorly defined (fuzzy) centrilobular nodules
subacute
135
what phase of hypersensitivity pneumonitis: fibrosis; interlobular and intralobular interstitial thickening, honeycombing and traction bronchiectasis
chronic
136
lobes mostly affected in hypersensitivity pneumonitis
mid to lower lung zones
137
radiographic feature of hypersensitivity pneumonitis that may help distinguish it from UIP
relative sparing of costophrenic angles in hypersensitivity pneumonitis
138
characterized histologically by noncaseating granulomas that may progress to fibrosis
sarcoidosis
139
detected in bronchoalveolar lavage in patients with sarcoidosis
excess number of T-helper lymphocytes
140
giant cells in the granulomas of sarcoidosis that may contain dark-staining lamellated structures within their cytoplasm
Schaumann bodies
141
approximately 90% involves this interstitium in the lung that makes blind transbronchial biopsy in sarcoidosis possible
axial interstitium
142
key feature in sarcoidosis that allows distinction from malignancy and TB
symmetric lymph node enlargement
143
most common parenchymal abnormality in sarcoidosis
bilateral symmetric reticulonodular opacities
144
usual lobes affected in sarcoidosis
mid and upper lung zones
145
Sarcoidosis stage: bilateral hilar lymph node enlargement
stage 1
146
sarcoidosis stage: bilateral hilar lymph node enlargement and parenchymal disease
stage 2
147
sarcoidosis stage: parenchymal disease only
stage 3
148
sarcoidosis stage: pulmonary fibrosis
stage 4
149
characteristic pulmonary function test finding in LCH
restrictive and obstructive patterns and a diminished diffusing capacity
150
systemic autoimmune disorder characterized pathologically by a necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts and kidneys
granulomatosis with polyangitis
151
characteristic lesions in the lungs are discrete nodules or masses of granulomatous inflammation with central necrosis and cavitation
granulomatosis with polyangitis
152
true or false: renal involvement follows respiratory involvement in 90% of patients with granulomatosis with polyangitis
true
153
treatment for granulomatosis with polyangitis
cyclophosphamide (cytoxan) and cotrimoxazole (Bactrim)
154
a transient pulmonary process characterized pathologiclly by pulmonary infiltration with an eosinophilic exudate. Most patients have a history of allergy, most commonly asthma
simple pulmonary eosinophilia aka Loffler syndrome
155
characteristic radiographic findings in Loffler syndrome
peripheral, homogeneous, ill-defined areas of airspace opacities that may parallel the chest wall
156
true or false: most patients with Loffler syndrome have self-limiting illness that resolves spontaneously within 4 weeks
true
157
idiopathic condition characterized by severe dyspnea and hypoxa lasting <5 days and > 25% eosinophils on pulmonary lavage. Progression is rapid as is resolution after steroid therapy
acute eosinophilic pneumonia
158
eosinophilic pneumonia is considered chronic after how many months with symptoms
1 month
159
chronic eosinophilic pneumonia has predilection for what gender
women
160
systemic disorder with a male predominance that is characterized by multiple organ damage from eosinophilic infiltration of tissues
hypereosinophilic syndrome
161
true or false: in hypereosinophilic syndrome, major chest findings are associated with cardiac involvement causing congestive heard failure: cardiomegaly, pulmonary edema and PE
true
162
drugs associated with pulmonary eosinophilia
nitrofurantoin and penicillin
163
parasitic infections most commonly attributed with pulmonary eosinophilia
filaria, ascaric lumbricoides and strongyloides stercoralis
164
predominant chest radiographic finding in polyarteritis nodosa is
hemorrhage caused by a vasculitis involving the bronchial arterial circulation
165
multisystem disorder in which asthma, blood eosinophilia, necrotizing vasculitis and extravascular granulomas are invariable features
allergic angitis and granulomatosis (Churg-Strauss syndrome)
166
major histologic principal patterns of drug-induced lung damage are
DAD, UIP, NSIP, bronchiolitis obliterans-organizing pneumonia, eosinophilic lung disease, and pulmonary hemorrhage
167
histologic patterns of drug induced lung damage that result from an acute lung insult
DAD, eosinophilic lung disease and pulmonary hemorrhage
168
histologic patterns of drug induced lung damage that result from an chronic lung toxicity
UIP, NSIP and BOOP
169
most commonly results from an acute insult to the lungs resulting in damage to type II pneumocytes and the alveolar endothelium
DAD
170
also referred to as chronic interstitial pneumonia when known to result from drug toxicitiy
NSIP
171
a relatively common result of pulmonary drug toxicity and usually responds well to cessation of therapy and steroid
OP
172
pulmonary hemorrhage in drug-induced pulmonary vasculitis is due to
complicate anticoagulation therapy or result from drug-induced thrombocytopenia
173
small airways inflammatory process that results in granulation tissue within bronchioles causing air trapping which can be severe enough to result in respiratory insufficiency
obliterative bronchiolitis
174
pulmonary toxicity in this drug that is used to treat acute leukemia develops in 15-30% of treated patients within 30 days of administration and is manifested as pulmonary edema resulting from increased capillary permeability
cytosine arabinoside (Ara-C)
175
rare disease in which lipoproteinaceous material surfactant deposits in abnormal amounts within the airspaces of the lungs. it has predilection for males in their 20s or 40s
pulmonary alveolar proteninosis
176
important clinical feature distinguishing PAP from pulmonary edema secondary to congestive heart failure
absence of orthopnea
177
patients with PAP are particularly prone to superinfection of the lung with
nocardia, aspergillus, cryptococcus and atypical mycobacteria
178
rare disorder characterized by the deposition of minute calculi within the alveolar spaces
alveolar microlithiases
179
true or false: there is very high incidence of alveolar microlithiasis in siblings
true
180
the radiographic findings in this rare entity include confluent bilateral dense micronodular opacities that, because of their high intrinsic density, produce the so-called "black pleural sign" at their interface with the chest wall. apical bullous disease is also common and may lead to spontaneous pneumothorax
alveolar microlithiasis
181
uncommon condition characterized by the formation of bone within the lung parenchyma
diffuse pulmonary ossification
182
nodular form of diffuse pulmonary ossification is seen in
severe, chronic untreated mitral stenosis
183
more irregular ossification in diffuse pulmonary ossification is seen in
chronic inflammatory conditions such amyloidosis and UIP
184
refers to a pattern of lung injury characterized by organizing pneumonia and fibrin within the alveolar space that does not meet strict histopatholohic criteria for DAD or organizing pneumonia
acute fibrinous and organizing pneumonia
185
clinical syndrome resulting from the coexistence of emphysema and pulmonary fibrosis, whether both secondary to smoking or of different etilogies
combined pulmonary fibrosis and emphysema syndrome
