what are clinically present in interstitial lung disease
restrictive lung disease and hypoxemia on pulmonary function tests
Scaffolding of the lung, providing support for the airways, gas-exchanging units and vascular structures
Pulmonary interstititum
The central interstitial compartment extending from the mediastinum peripherally and enveloping the bronchovascular bundles is termed the
Axial interstitium
Axial interstitium is contiguous with the interstitium surrounding the small centrilobular arterieand bronchiole within the secondary pulmonary lobule, where it is called
Centrilobular intersititium
The most peripheral component of the interstitium is the
Subpleural or peripheral interstitium
Invaginations of the subpleural interstitium into the lung parenchyma from the borders of the secondary pulmonary lobules and represent the
Interlobular septa
fine network of connective tissue fibers that support the alveolar spaces
intralobular, parenchymal or alveolar interstitium
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)
secondary pulmonary lobule
each terminal bronchiole subdivide into
respiratory bronchioles, alveolar ducts, alveolar sacs and alveoli
unit of lung subtended from a single terminal bronchiole is called a
pulmonary acinus
located in the center of secondary lobule
centrilobular artery, preterminal bronchiole
present at the margins of lobules within the interlobular septa, found within the contiguous subpleural interstitium
lymphatic and connective tissue
secondary pulmonary lobule is typically of what shape
polyhedral
normally 0.1 mm thick and can be seen in lung periphery, particularly along the superior and inferior pleural surfaces
interlobular septa
are V- or Y- shaped structures on thin-section CT seen within 5 to 10 mm of pleural surface
centrilobular arteries
normal airways are visible only to within ___cm of the pleura
3cm
has a diameter of 1mm and a wall thickness of 0.15 mm, not normally visible on thin-section CT
centrilobular brochiole
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
pulmonary veins
normally not visible on thin section CT
peribronchovascular, centrilobular, and intralobular interstitial compartments
interlobular lines on thin-section CT are equivalent of _____ lines seen in the inferolateral portions of the lungs on frontal radiographs
Kerley B
within the central regions of the lung, long (2-6 cm) linear opacities representing obliquely oriented connective tissue septa are the equivalent of ______ lines
Kerley A
thickened intralobular lines are usually from
fibrosis, UIP, IPF
seen within the central portion of pulmonary lobule radiating outward the thickened lobular borders to produce a “spoke-and-wheel” or “spider web” appearance
intralobular septa
nodular fissural thickening are usually seen in
sarcoidosis and lympangitic carcinomatosis
can either result in smooth or irregular peribronchovascular thickening
lymphangitic carcinomatosis
centrilobular abnormalities are usually seen in
subacute hypersensitive pneumonitis, cryptogenic organizing pneumonia, respiratory bronchiolitis-associated interstitial lung disease
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
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
Nontapering linear opacities 2-5 cm in length, that extend from the lung to contact pleural surface
Parenchymal bands
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
Area of increased attenuation within which the normal parenchymal structures are visible
Ground glass opacity
1-3 mm sharply marginated round opacities seen on thin section CT which represent conglomerates of granulomas or tumor cells within the interstitium
Micronodules
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
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
Ground glass abnormality associated with a predominant pattern of honeycombing indicates
Microscopic pulmonary fibrosis
Finding commonly associated with architectural distortion is
Traction bronchiectasis
Refers to increased lung density that obscures underlying blood vessels; air bronchograms are commonly present
Consolidation
Results from diffuse inflammatory processes that primarily affect the axial and parenchymal interstitium of the lung
Chronic interstitial disease
Chronic interstitial pulmonary edema are commonly seen in patients with
Long standing mitral stenosis, or LV failure
Redistribution of blood flow to the upper lobes is a manifestation of
Pulmonary venous hypertension
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
Pulmonary involvement in RA is more common in what gender
Male
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
Most common radiographic manifestation of RA lung involvement
Interstitial pneumonitis and fibrosis
Most common thoracic manifestation of rheumatoid disease and is found in 20% of patients
Pleuritis
Pleural effusions in RA are transudative or exudative
Exudative, with low glucose concentration
May develop from diffuse interstitial fibrosis secondary to rheumatoid disease
Enlargement of central pulmonary arteries and right heart dilatation
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
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
In SLE, pleura and pericardium exhibits _____ which produces painful exudative pleural and pericardial effusions
Fibrinous serositis
Results in diffuse pleural thickening and is present in the majority of patients with long standing SLE
Pleural fibrosis
Pleural effusions in SLE typically improves after what tx
Corticosteroid therapy
Characterized by rapid onset of fever, dyspnea and hypoxemia, which may require ventilation in patients with SLE
Acute lupus pneumonitis
Produces inflammation and fibrosis of the skin, esophagus, msk, heart, lungs and kidneys in young and middle-aged women
Scleroderma
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
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
Pulmonary arterial hypertension in patients with scleroderma results from
Thickening and obliteration of small muscular pulmonary arteries and arterioles
True or false, pleural effusions are significantly less common in scleroderma than in rheumatoid disease or SLE
True
Common lung ca that may be present with scleroderma
Bronchoalveolar cell carcinoma
Involve autoimmune inflammation and destruction of skeletal muscle, producing proximal muscle pain and weakness and occassionally associated with a skin radh
Dermatomyositis and polymyositis
Lung ca that is mostly seen in patietns with dermatomyositis or polymyositis
Bronchogenic carcinoma
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
Components of sicca syndrome
Dry eyes (keratoconjunctivitis sicca), dry mouth (xerostamia), dry nose (xerorhinia)
Patients with Sjogren syndrome are at increased risk for developing
Lymphocytic interstitial pneumonitis and non-hodgkin pulmonary lymphoma
Possible Lung findings in ankylosing spondylitis
Upper lobe pulmonary fibrosis, bullae and cavities which are prone to mycetoma formation with aspergillus
Most common form of IIP
Usual IP
Distinguishing histologic feature of UIP
Different stages of the disease are seen simultaneously within different portions of the lung (temporal heterogeneity)
Pulmonary function tests in UIP presents as
Restrictive disease, and a decreased diffusing capacity of the lungs for carbon monoxide
Age of onset and gender predilection of UIP
5th to 7th decades and male predominance
Distribution of UIP
Basal predominant, subpleural predominant
Most common histologic type of lung cancer in IPF
Adenocarcinoma
Many cases of NSIP are seen associated with
Collagen vascular disease or as drug reactions
Distribution of ground glass opacities and consolidation in NSIP
Peripheral and lower zone distribution
Honeycombing is seen frequently in UIP or NSIP?
UIP
Distribution of ggo, small centrilobular nodules in respiratory bronchiolitis associated interstitial pneumonia
Upper lobe
Usual concomitant finding in respiratory bronchiolitis-associated interstitial lung disease
Emphysema
Interstitial pneumonia characterized by accumulation of macrophages within alveolar spaces
Desquamative interstitial pneumonia
IP usually seen in smokers
DIP
Typical radiographic findings in DIP
Basilar reticular opacities with normal or minimally diminished lung volumes, mostly often within the peripheral aspects of the bases
Characterized by the widespread deposition of granulation tissue (fibroblasts,collagen and capillaries) within peribronchiolar airspaces and bronchioles
Organizing pneumonia or COP
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
Characteristic histologic changes in COP
Absence of parenchymal distortion and fibrosis
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
Rare, potentially fatal disease characterized by elastic tissue-rich fibrosis involving the pleura and adjacent lung parenchyma
Pleuroparenchymal fibroelastosis
Most severely involved lobe in pleuroparenchymal fibroelastosis
Upper lobes
Thoracic manifestations of NF1
Kyphoscoliosis, scalloping of posterior aspect of vertebral bodies caused by dural ectasia, ribbon rib and rib notching
Mediastinal masses in patients with NF1 include
Neurofibromas, lateral thoracic meningoceles, extra-adrenal pheochromocytomas
Classic triad of tuberous sclerosis
Seizures, mental retardation and adenoma sebaceum
Lymphangioleiomyomatosis is an uncommon condition that is seen exclusively in
Women
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
Drug that may slow the progression of LAM
Antiprogesterone agents such as tamoxifen
Type of alveolar septal amyloidosis in which there is no associated chronic disease or in which there is an underlying plasma cell disorder
Primary
Type of alveolar septal amyloidosis where there is an underlying chronic abnormality such as TB
Secondary
Type of amyloidosis that is usually localized to nervous tissue
Familial
Three major patterns of amyloid deposition within the lungs and airways
Tracheobronchial, nodular parenchymal, diffuse parenchymal (alveolar septal)
Amyloid is deposited in the parenchymal interstitium and within the media of small blood vessels
Alveolar septal amyloidosis
True or false. There is no effective treatment in alveolar septal amyloidosis
True
Lung biopsy findings in amyloidosia
Amorphous eosinophilic material thickening the alveolar septa that appears apple green in color when stained with congo redo
Used to describe the nonneoplastic reaction of the lungs to inhaled inorganic dust particles
Pneumoconiosis
2 types of pathologic reaction in pneumoconiosis
Fibrosis and aggregation of particle-laden macrophages
What type of asbestos have a greater fibrogenic and carcinoenic potential
Amphiboles
Most common disease associated with asbestosis
Pleural disease, as parietal pleural plaques
Pulmonary parenchymal manifestations of asbestosis
Rounded atelectasis, interstitial fibrosis, and bronchogenic carcinoma
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
Most common ct finding in asbestos-exposed individuals
Interlobular septal thickening
2 histopathologic reaction to silicosis
Silicotic nodules and silicoproteinosis
Silicotic nodules measures ___ and are made up of dense concentric lamellae of collagen
1-10 mm
Silicotic nodules are typically most numerous in what regions
Upper lobes and parahilar regions
Exposure of how many years before radiographic changes of fibrotic silicosis develops
10-20 years
Hilar nodes in silicosis often demonstrate
Eggshell calcification
Patients with acute silicoproteinosis have an increased susceptibility to
TB and nocardia
2 characteristic pathologic finding in CWP
Coal dust macule and PMF
Round or stellate nodules ranging in size from 1-5mm seen in cwp
Coal dust macule
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
PMF in cwp are often seen in what lung segments
Posterior segments of upper lobes and superior segments of lower lobes
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
Pneumoconiosis that produces a reaction that mimics sarcoidosis
Chronic berylliosis
Extrinsic allergic alveolitis is an immunologic pulmonary disorder associated with the inhalation of one of the antigenic organic dusts
Hypersensitivity pneumonitis
Exposure to moldy hay produce
Farmer’s lung
Exposure to water reservoirs contaminated by thermophilic bacteria
Humidifier lung
Exposure to avian proteins in feathers and excreta produce
Bird fancier’s lung
What type of hypersensitivity reaction is produced in hypersensitivity pneumonitis
Type 3 (immune complex) reaction
Radiographic features of early hypersensitivity pneumonitis
Fine nodular or ground glass opacities most often seen in lower lobes
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
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
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
In the mid-lung, where the bronchi course horizontally, the appearance of bronchiectasis is that of parallel linear opacities called
Tram tracks
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
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
True or false: distal atelectasis and obstructive pneumonitis are common findings in cystic fibrosis
True
Disorder in which the epithelial cilial motion is abnormal and ineffective
Primary ciliary dyskinesia aka dysmotile cilia syndrome
May result in rhinitis, sinusitis,bronchiectasis, dysmotile spermatozoa and sterility, situs inversus, and dextrocardia
Primary ciliary dyskinesia
what phase of hypersensitivity pneumonitis: airspace opacities
acute
what phase of hypersensitivity pneumonitis: patchy areas of ggo and poorly defined (fuzzy) centrilobular nodules
subacute
what phase of hypersensitivity pneumonitis: fibrosis; interlobular and intralobular interstitial thickening, honeycombing and traction bronchiectasis
chronic
lobes mostly affected in hypersensitivity pneumonitis
mid to lower lung zones
radiographic feature of hypersensitivity pneumonitis that may help distinguish it from UIP
relative sparing of costophrenic angles in hypersensitivity pneumonitis
characterized histologically by noncaseating granulomas that may progress to fibrosis
sarcoidosis
detected in bronchoalveolar lavage in patients with sarcoidosis
excess number of T-helper lymphocytes
giant cells in the granulomas of sarcoidosis that may contain dark-staining lamellated structures within their cytoplasm
Schaumann bodies
approximately 90% involves this interstitium in the lung that makes blind transbronchial biopsy in sarcoidosis possible
axial interstitium
key feature in sarcoidosis that allows distinction from malignancy and TB
symmetric lymph node enlargement
most common parenchymal abnormality in sarcoidosis
bilateral symmetric reticulonodular opacities
usual lobes affected in sarcoidosis
mid and upper lung zones
Sarcoidosis stage: bilateral hilar lymph node enlargement
stage 1
sarcoidosis stage: bilateral hilar lymph node enlargement and parenchymal disease
stage 2
sarcoidosis stage: parenchymal disease only
stage 3
sarcoidosis stage: pulmonary fibrosis
stage 4
characteristic pulmonary function test finding in LCH
restrictive and obstructive patterns and a diminished diffusing capacity
systemic autoimmune disorder characterized pathologically by a necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts and kidneys
granulomatosis with polyangitis
characteristic lesions in the lungs are discrete nodules or masses of granulomatous inflammation with central necrosis and cavitation
granulomatosis with polyangitis
true or false: renal involvement follows respiratory involvement in 90% of patients with granulomatosis with polyangitis
true
treatment for granulomatosis with polyangitis
cyclophosphamide (cytoxan) and cotrimoxazole (Bactrim)
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
characteristic radiographic findings in Loffler syndrome
peripheral, homogeneous, ill-defined areas of airspace opacities that may parallel the chest wall
true or false: most patients with Loffler syndrome have self-limiting illness that resolves spontaneously within 4 weeks
true
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
eosinophilic pneumonia is considered chronic after how many months with symptoms
1 month
chronic eosinophilic pneumonia has predilection for what gender
women
systemic disorder with a male predominance that is characterized by multiple organ damage from eosinophilic infiltration of tissues
hypereosinophilic syndrome
true or false: in hypereosinophilic syndrome, major chest findings are associated with cardiac involvement causing congestive heard failure: cardiomegaly, pulmonary edema and PE
true
drugs associated with pulmonary eosinophilia
nitrofurantoin and penicillin
parasitic infections most commonly attributed with pulmonary eosinophilia
filaria, ascaric lumbricoides and strongyloides stercoralis
predominant chest radiographic finding in polyarteritis nodosa is
hemorrhage caused by a vasculitis involving the bronchial arterial circulation
multisystem disorder in which asthma, blood eosinophilia, necrotizing vasculitis and extravascular granulomas are invariable features
allergic angitis and granulomatosis (Churg-Strauss syndrome)
major histologic principal patterns of drug-induced lung damage are
DAD, UIP, NSIP, bronchiolitis obliterans-organizing pneumonia, eosinophilic lung disease, and pulmonary hemorrhage
histologic patterns of drug induced lung damage that result from an acute lung insult
DAD, eosinophilic lung disease and pulmonary hemorrhage
histologic patterns of drug induced lung damage that result from an chronic lung toxicity
UIP, NSIP and BOOP
most commonly results from an acute insult to the lungs resulting in damage to type II pneumocytes and the alveolar endothelium
DAD
also referred to as chronic interstitial pneumonia when known to result from drug toxicitiy
NSIP
a relatively common result of pulmonary drug toxicity and usually responds well to cessation of therapy and steroid
OP
pulmonary hemorrhage in drug-induced pulmonary vasculitis is due to
complicate anticoagulation therapy or result from drug-induced thrombocytopenia
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
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)
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
important clinical feature distinguishing PAP from pulmonary edema secondary to congestive heart failure
absence of orthopnea
patients with PAP are particularly prone to superinfection of the lung with
nocardia, aspergillus, cryptococcus and atypical mycobacteria
rare disorder characterized by the deposition of minute calculi within the alveolar spaces
alveolar microlithiases
true or false: there is very high incidence of alveolar microlithiasis in siblings
true
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
uncommon condition characterized by the formation of bone within the lung parenchyma
diffuse pulmonary ossification
nodular form of diffuse pulmonary ossification is seen in
severe, chronic untreated mitral stenosis
more irregular ossification in diffuse pulmonary ossification is seen in
chronic inflammatory conditions such amyloidosis and UIP
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
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
