Diffuse Lung disease Flashcards
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 (3)
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 (3)
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
- fibrosis causes traction on the walls of bronchi, resulting in irregular dilatation.
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
pulmonary arterial hypertension
Chest wall abnormalities that may be seen with RA (4)
- 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 (2)
Collagen vascular disease or as drug reactions
Distribution of ground glass opacities and consolidation in NSIP
Peripheral and lower zone distribution
- cellular NSIP show areas of ground glass and consolidation on thin-section CT in a peripheral and lower zone distribution
Bronchial dilatation and linear opacities are more typical of the fibrotic form of NSIP
Honeycombing is seen frequently in UIP or NSIP?
UIP