pulmonology3 Flashcards
Group 1 pulmonary hypertension
this is pulmonary arterial hypertention causes include: Idiopathic PAH, Heritable PAH, Drug and toxin induced and can be Associated with: Connective tissue disease, HIV infection, Portal hypertension, Congenital heart diseases, and Schistosomiasis
Group 2 pulmonary hypertension
this is pulmonary hypertension due to left heart disease and can be caused by: left ventricular systolic dysfunction, Left ventricular diastolic dysfunction, Valvular disease, and Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies.
Group 3 pulmonary hypertension
this is pulmonary hypertension due to lung disease and/or hypoxia and includes: Chronic obstructive pulmonary disease, Interstitial lung disease, Other pulmonary diseases with mixed restrictive and obstructive pattern, Sleep-disordered breathing, Alveolar hypoventilation disorders, Chronic exposure to high altitude, and Developmental lung diseases
Group 4 pulmonary hypertension
Chronic thromboembolic pulmonary hypertension (CTEPH)
Group 5 pulmonary hypertension
Pulmonary hypertension with unclear multifactorial mechanisms and includes: Hematologic disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy, Systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis, Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders, and Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH
UIP – Usual Interstitial Pneumonitis (or Pneumonia)
The histopathologic pattern seen in Idiopathic Pulmonary Fibrosis (and other diseases).
IPF – Idiopathic Pulmonary Fibrosis
a clinical diagnosis of idiopathic (or unexplained) usual interstitial pneumonia.
Inexorable progression, 2-3 year survival, unresponsive to therapy. Smoking is a risk factor. More common in elderly. Radiographic findings include Basilar and peripheral predominant reticular abnormality, traction bronchiectasis, volume loss, honeycombing, paucity of ground glass. Histopathological findings of Spatially and temporally heterogeneous fibrosis with fibroblast foci and little inflammation (UIP pattern)
NSIP – Nonspecific Interstitial Pneumonitis (or Pneumonia)
Better prognosis than IPF, the more cellular, the more steroid responsive. Radiographic findings include basilar and peripheral predominant reticular abnormality, traction bronchiectasis, volume loss, usually with associated ground glass. Histopathological findings include spatially and temporally homogeneous fibrosis. Cellular and fibrotic variants.
RB-ILD – Respiratory Bronchiolitis
Interstitial Lung Disease. Smoking related; responds to smoking cessation, variably steroid responsive. Radiographic findings include airway thickening with centrilobular nodules, gas trapping, and patchy ground glass opacity. Histopathological findings include bronchiolocentric accumulation of “dusty brown” macrophages with peribronchiolar lymphocitic and monocytic infiltrates and peribronchiolar fibrosis.
DIP – Desquamative Interstitial Pneumonitis (or Pneumonia)
a misnomer based on the incorrect initial hypothesis that the cells filling the alveolar spaces were desquamated epithelial cells; these are actually alveolar macrophages (also a misnomer in that it is not a purely interstitial process. alveolar filling is the primary process, though there is also expansion of the alveolar septa and pulmonary interstitium). Smoking related; maybe on a spectrum with RB- ILD, responds to smoking cessation and variably to corticosteroids. Radiographic findings include patchy, basilar predominant ground glass opacity, sometimes with irregular reticular abnormality. Histopathological findings include thickened alveolar septa with “dusty brown” macrophages filling distal airspaces
DAD – Diffuse Alveolar Damage
the histopathologic pattern seen in AIP (and in other processes such as ARDS and some drug toxicities). Also called “acute lung injury.”
AIP – Acute Interstitial Pneumonitis (or Pneumonia)
similar to DIP, the primary process in AIP - and other entities marked by DAD - is alveolar filling. Rapid progression to respiratory failure and death, poorly responsive to therapy. Radiographic findings include diffuse alveolar filling with patchy ground glass, septal thickening, and traction bronchiectasis. Histological findings include alveolar edema, neutrophils, hemorrhage, hyaline membranes (similar pattern as ARDS). Diffuse alveolar damage (DAD) pattern of injury
COP – Cryptogenic Organizing Pneumonia
(of note, Organizing Pneumonia may also be seen in non-cryptogenic processes such as collagen-vascular disease and drug toxicity – COP refers specifically to Organizing Pneumonia without an evident precipitant). Subacute presentation, may be associated with collagen vascular dz, malignancy, infection, exposures, or idiopathic. Steroid responsive. Radiographic findings include bilateral peripheral alveolar opacities (fuzzy nodules) with preserved lung volumes. May be migratory. Thickened airways. Histopathological findings include intraluminal plugs of granulation tissue with peribronchiolar inflammation and sometimes intra- alveolar neutrophils.
LIP – Lymphoid (or Lymphocytic) Interstitial Pneumonitis (or Pneumonia)
Associated with immunodeficiency, Sjögren’s, lymphoma, or idiopathic. Radiographic findings include centrilobular nodules, ground glass sometimes, cyst formation in end stage disease. Histopathological findings include lymphocytic peribronchial and alveolar septal infiltrates, sometimes with germinal centers
AEP – Acute Eosinophilic Pneumonitis (or Pneumonia)
Presentation indistinguishable from DAD or pulmonary edema. Often febrile prodrome. AEP, however responds to steroids. Can mimic ARDS. Radiographic findings include diffuse bilateral alveolar infiltrates, indistinguishable from pulmonary edema, DAD, or ARDS. Histopathological findings is similar to DAD, though with intra-alveolar and septal eosinophilic infiltrates
CEP – Chronic Eosinophilic Pneumonitis (or Pneumonia)
Subacute presentation, constitutional sx, more in nonsmokers and women, steroid responsive. Radiographic findings are described as “Photographic negative” of pulmonary edema. Peripheral ground glass and reticular opacity. Histopathological findings include alveolar septal thickening, with eosinophilic infiltrates, fibrosis, macrophages
LCH – Langerhans Cell Histocytosis
formerly (and sometimes still) referred to as Eosinophilic Granuloma (but no eosinophils and no granulomas!) or EG. Smoking-related, younger patients, spontaneous pneumothorax in 25%. Radiographic findings include irregular cysts and nodules, upper zone predominant, spares costophrenic angles. Histopathological findings show Infiltration of Langerhans cells
LAM – Lymphangioleiomyomatosis
Women only, may respond to anti-estrogen therapy. Obstructive PFTs; pleural effusion (chylothorax) and spontaneous pneumothax common. Radiographic findings cysts and nodules, in a more random shape, size and distribution than in LCH. Pleural effusions and PTX common. Histopathological findings show peribronchovascular proliferation of smooth muscle cells, lymphatic occlusion
HP—Hypersensitivity pneumonitis
An immunologic response to inhaled organic antigen (mold, bird proteins most common). May be acute or chronic. Chronic disease is often fibrotic. Antigen avoidance key. Radiographic findings: Acute: centrilobular ground glass
Chronic: reticular pattern with fibrosis
Both are often upper-lobe predominant with mosaic attenuation. Histopathological findings show inflammation +/- fibrosis with poorly formed granulomas
“Interstitial” lung diseases with a prominent alveolar filling component
DIP, AIP, Organizing Pneumonia, AEP, CEP
Silhouette sign
is somewhat of a misnomer and in the true sense actually denotes the loss of a silhouette, thus it is sometimes also known asloss of silhouette signorloss of outlinesign. The differential attenuation of x-ray photons by two adjacent structures defines the silhouette, e.g. heart borders against the adjacent lung segments and it is the pathological loss of this differentiation, which the silhouette sign refers to.
Air bronchogram
refers to thephenomenonofair-filled bronchi (dark) being made visible by the opacification of surrounding alveoli (grey/white). It is almost always caused by a pathologic airspace/alveolar process, in which something other than air fills the alveoli.Air bronchograms will not be visible if the bronchi themselves are opacified(e.g. by fluid) and thus indicate patent proximal airways.
Subcutaneous emphysema
strictly speaking, refers to air in the subcutaneous tissues. But the term is generally used to describe any soft tissue emphysema of the body wall or limbs, since the air often dissects into the deeper soft tissue and musculature along fascial planes.
Producing a chest radiographic image
X-rays are produced by bombarding a rotating tungsten target with a focused electron beam. Even though x-rays are in the non-visible high-energy electromagnetic spectrum, the x-rays emit a divergent beam much like a flashlight from a focal point in the direction of the radiograph detector. The patient casts a shadow on the detector that becomes the radiograph. Thoracic anatomy has better resolution as it becomes farther away from the focal point (radiation source). As well, there is less magnification of the various anatomy when it is located further away from the focal point. This concept can be reproduced when taking a flashlight and making hand shadows at various distances between the flashlight and the wall. Therefore, anatomy such as the heart will appear larger on the radiograph if it is closer to the source and further from the detector (as in the source is anterior and the detector is posterior to the chest, termed “AP view.”). For this reason, ideal positioning of the patient for standard two-view chest radiographs places their anterior chest against the detector for the frontal view and the left chest against detector for the lateral view. The divergent beam/magnification also affects the appearances of other anatomy in the chest, such as ribs.