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42 Cards in this Set
- Front
- Back
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What is interstitium?
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B.M. of endothelial/epithelial cells
Collagen Elastic tissue Fibroblasts Few mast cells occasional Lymphs/monos Proteoglycans |
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What is the classical feature of Restrictive Lung Disease?
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reduction in
COM diffusing capacity lung vol compliance |
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What will Chest radiograph show in RLD?
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bilateral infiltrate lesions
small nudules irregular lines or ground-glass shadows |
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What does TGF-beta 1 control in IPF?
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Telomerase activity - facilitates apoptosis
Caveolin-1 - inhibitor of pulmonary fibrosis - lack -> extracellular matrix production |
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What is the earliest lesion in UIP?
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fibroblastic foci - fibroblast/myofibroblast
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How would you stain for fibrosis (collagen)?
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Trichome
Collagen stains blue |
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What is the common manifestation of the interstitial fibrosis in the lungs?
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Alveolitis
Accumulation of inflammatoryu and immune effector cells within alveolar walls and spaces |
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What are the 2 effects of leukocytes in fibrosis?
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distortion of normal alveolar structures
results in release of mediators (stim parenchymal cells and stim fibrosis) |
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What is Micro Hallmark for UIP (usual interstitial pneumonia?
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patchy interstitial fibrosis
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What is honeycomb fibrosis in UIP?
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destroyed alveolar structures and formation of cystic spaces are lined with type II pneumocytes or bronchiolar epithelium
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What are the significant feature of DIP (Desquamative Interstitial Pneumonia)?
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Most striking feature - accumulation of macrophages with dusty brown pigment in the airspaces
Lamellar bodies - contain surfactnat Lined with plump, cuboidal pneumocytes emphysema minimal fibrosis mild restrictive PFT's signs non-progressive |
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What is the main feature of RB-ILD (respiratory bronchiolitis-interstitial lung disease)?
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accumulation is centroacinar vs. diffuse as in DIP
w/in respiratory bronchioles resp. bronchioles, alveolar ducts, peribronchial spaces - dusty macrophages patchy at low magnification mild fibrosis |
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What is the most significant about AIP (acute Interstitial pneumonia)?
aka Hamman-Rich syndrome |
rapid progression
brisk interstitial fibroblastic proliferation resembles ARDS (rad and physio) and equivalent to that of organizing phase of DAD diffuse bilateral airspace opacification if recovered - complete recovery of PF |
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What is the most dangerous size of particles?
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1.0 - 5.0 micro meters in diameter
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What is unique about silica?
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silica w/in macrophages activates them
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What are the ranges/differences in Coal Worker's Pneumoconiosis?
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1. Anthracosis - carbon pigment inhaled -> engulfed by alveolar or interstitial macrophages -> accumulated near lympathics (lymphoid tissue along bronchi/lung hilus)
not fibrogenic pigment 2. Simple CWP - coal macules (carbon-laden macrophages) small amount collagen fibers upper lobes/upper zones next to resp. bronchioles dilation of adjacent alveoli little to no change in pulm fx 3. Complicated CWP/PMF mild form - no great loss of pulm fx large blackened scars in upper lobes extensions into parenchyma dense collagen and pigment center - necrotic/d/t local ischemia |
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What are the forms of silica and which one is more fibrogenic?
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crystalline and amorphous
Crystalline - more fibrogenic contains quartz, crystobalite, and tridymite |
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Which particle is most implicated in silicosis? And what is effect in mixtures?
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Quartz
reduces fibrogenic effect |
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How to distinguish acute silicosis?
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accumulation of lipoprotenacoeus material w/in alveoli
morphology similar to PAP - IHC - sufarctant protein A, B, C PAS + contains cholesterol clefts nodules may not be present happens after inhalation of high doses of fine silica particles |
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What is the characteristic feature in acute silicosis?
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Large multilamellated structures
composed of tubular myelin-like membranes |
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Describe 2 different forms of asbestos
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Serpentine - Curly and flexible fibers
Amphibole - straight, stiff, and brittle fibers; more pathogenic Both are fibrogenic |
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Which form of asbestos associated with mesothelioma?
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amphibole exposure
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What is asbestosis?
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diffuse pulmonary interstitial fibrosis with asbestos bodies
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How do asbestos bodies look like?
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golden brown, fusiform or beaded rods;
Translucent center Coated wtih Fe-containing proteinaceous material Looks like dumbbells |
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What is the most common manifestation of asbestos exposure?
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pleural plaques
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Describe pleural plaques
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Well-circumscribed of dense collagen
often contains Ca frequently on anterior and posteriorlateral aspects DO NOT contain asbestos bodies |
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Where do typically asbestos bodies show up?
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pleura and subjacent lung
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Describe sarcoidosis
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non-caseating granulomas in tissues and organs
bilateral hilar lymphadenopathy or lung involvement 90% visible on CXR W>M and more in AA Common sites - Lung Macro - no demonstrable alterations Micro - along the lymphatics, around bronchi, blood vessels, occ. alveolar lesions |
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What is Nijmegan Breakage Syndrome?
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the mutation of NBS1 gene on chrom 8q21
chromosomal instability congenital, autosomal recessive shows up as - short stature, distinct facial appearance, immunodeficiency, predisposition to lymphoid malignancy cutaneous sarcoidosis |
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What is the pathogenesis of sarcoidosis?
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disease of disordered immune regulation in genetically predispose person
genetic factors - class I HLA-A1 and HLA-B8 |
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Describe the morphology of sarcoidosis
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all tissues - non-caseating granulomas
aggregates of epitheliod/epithelioid histiocytes often Langhans or giant cells Central necrosis UNUSUAL Schaumann bodies - Ca+ protein Asteroid bodies |
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What is Schaumann Body?
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basophilic, lamellated shell like
in cytoplasm of multinucleated giant cells polarized light - birefringent can also be found extracellularly Common in Sarcoidosis, but non-specific |
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What are asteroid bodies?
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cytoplasmic inclusions in giant cells of granulomas
star-like, spider, or umbrella |
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What is Allergic Bronchopulmonary Aspergillosis/ABPA?
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results from hypersensitivity rxn to fungus Asperigillus fumigatus
complication of asthma and cystic fibrosis organisms are in the mucus or within mucous plugs Intense airway inflammation eosinophils formation of mucus plugs |
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What is Charcot-Leyden crystals?
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microscopic crystals found in people with allergic disease such as ABPA
crystal proteins interact with eosinophils and lysophospholipases |
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What is Chronic Eosinophilic Pneumonia? CEP
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generally rxn to drugs or fungi (most often Aspergillus)
d/t prolonged febrile illness peripheral eosinophilia is common CXR/CT - bilateral patchy infiltrates more in peripheral lungs Bronchiolitis obliterans can be present in CEP occ. infiltration of blood vessels -> appearance of vasculitis necrosis is not seen |
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Microscopic CEO description
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patchy and exudative
pneumonic consolidation intraalveolar exudate - large eosinophils, lymphs, plasma cells, macrophages some proteinaceous exudate and edema |
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What is Goodpasture's syndrome?
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uncommon autoimmune disease
present of autoantibodies targeting non-collagenous domain of alpha-3 chain of collagen IV Destruction of B.M. in glomeruli and alveoli necrotizing hemorrhagic interstitial pneumonitis |
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What is possible pathogenesis of Goodpasture's Syndrome?
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initial trigger unknown
genetics - HLA-DRB1 1501 and 1502 |
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How to distinguish GS and Idiopathic pulmonary hemosiderosis?
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looks like GS histologically, but NO Ab's , NO kidney disease
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Classical Morpholgy of Goodpasture's Syndrome
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Gross - lungs heavy, areas of red-brown consolidations
Micro - focal necrosis of alveolar walls often hemosiderin-laden macrophages in alveoli Later - fibrous thickening of septae; hypertrophy of type II pneumocytes IHC - linear deposits of Ig's along the basement membranes of septal walls |
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Clinical features of GS
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respiratory symptoms
hemoptysis CXR - focal pulmonary consolidations kidney failure - d/t uremia use of immunosupressive drugs prevent formation of Ab's |
