Pulmo Part 2 Flashcards by Denisse Tiangco

Hallmark of CILD (restrictive, infiltrative)

Reduced compliance (more pressure required to expand lungs bec they are stiff) necessitating effort in breathing (dyspnea)

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Unknown etiology
Patchy progressive bilateral interstitial fibrosis resulting in hypoxemia and cyanosis

Histology: usual intersitial pneumonia

Idiopathic pulmonary fibrosis

Cryptogenic fibrosing alveolitis

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Endogenous inhibitor of pulmonary fibrosis

Caveolin-1

Downregulated by TGFB1

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Cobblestones of pleural surface bec of retraction scars along interlobular septa
Patchy interstitial fibrosis
Fibroblastic foci
Temporal homogeneity (early and late lesion)
Honeycomb fibrosis

Idiopathic pulmonary fibrosis

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Dense fibrosis causing collapse of alveolar wall and cystic spaces lined by hyperplastic type II pneumocytes or bronchiolar epithelium

Honeycomb fibrosis

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Dry velcro like crackles on inspiration
Cyanosis, cor pulmonale
Peripheral edema
Lung biopsy for diagnosis

Mean survival 3 years

Tx: lung transplant

Idiopathic pulmonary fibrosis

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Mild to mod chronic interstitial inflamm in uniform or patchy distribution

Nonspecific interstitial pneumonia cellular

Best prognosis

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Diffuse patchy intersitial fibrosis without temporal homogeneity of UIP

Nonspecific intersitial pneumonia fibrosing

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BOOP Bronchiolitis obliterans organizing pneumonia
subpleural or perobronchial patchy areas of air space consolidation
Polypoid plugs of loose CT
Lung architecture normal
Tx: steroid 6 mos

Cryptogenic organizing pneumonia

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Cryptogenic organizing pneumonia histologic hallmark

Balls of fibroblast in alveolar space

Masson body

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Restrictive lung diseases with reduced lung compliance and reduced FVC

Ratio of FEV/FVC = normal

Diffuse interstitial fibrosis

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Unifying pathogenic factor of DIF

mac activation and TGFB release (fibrogenic)

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Patchy intersitial fibrosis, fibroblastic foci, cystic space formation honeycomb lung and Usual Interstitial Pneumonia

Idiopathic pulmonary fibrosis

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Non neoplastic lung reaction to mineral dust

Pneumoconiosis

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Particles 5-10 um are

Particles 0.5 less are

unable to reach distal airway

move into and out of alveoli without deposition

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Most dangerous bec they get lodged at the bifurcation of distal airway

1–5 um

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Key cellular element in initiation and perpetuation of lung injury and fibrosis

alveolar macrophage

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Smoking worsens effects of inhaled mineral dusts especially

asbestos

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Most common mineral dust pneumoconioses

coal
dust
silica

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Coal worker’s pneumoconiosis:

Macule and nodule

Coal mining

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Complicated coal worker’s pneumoconiosis

PMF

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Sandblast quarrying mining stone cutting foundry work, ceramics

Silicosis

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Pleural effusion, pleural plaque, diffuse fibrosis, mesothelioma, carcinoma of lung and larynx

Mining, milling, fabrication of ores and materials, installation and removal of insulation

Asbestos

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Pigment accum without perceptible cellular reaction

Anthracosis

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Most inoccuous coal induced pulmo lesion in coal miner and smoker Carbon engulfed mac accumulating in CT along lymphatics

Pulmonary anthracosis

Dust laden mac small amounts of collagen fibers arrayed in delicate network Frequent in upper lobes Centrilobular emphysema

Coal macule Coal nodule Simple CWP

Coalescence of coal nodule Multiple intense blackened scar larger than 2cm Dense collagen and pigment

Complicated CWP | Progressive massive fibrosis

Pulmonary dysfunction Pulmonary hypertension Cor pulmonale

Progressive massive fibrosis

Most prevalent chronic occupational disease worldwide

Silicosis

Most implicated in silicosis Less fibrosis if mixed with other minerals

quartz

Tiny barely palpable discrete pale-to-blackened nodules in upper zones of lungs Concentrically arranged hyalinized collagen fibers surrounding amorphous center Whorled appearance, weakly birefringent silica at the center sometimes with egg shell calcification in LN

Silicotic nodule

Silicosis is associated with inc susceptibility to

tuberculosis and lung cancer inhibited ability of mac to kill phagocystosed bacteria

Asbestos fiber is curly and flexible Most common and impacted at upper respiratory passage

Serpentine Chrysotile

Asbestos fiber is straight, stiff and brittle More pathogenic bec can lodge deep to interstitium

Amphibole

asbestos can produce

asbestosis lung ca mesothelioma

Important to synergy between smoking and lung ca in asbestos

adsorption of carcinogen in tobacco smoke onto asbestos

Golden brown fusiform rod with transluscent center | Asbestos fiber coated with iron material

Asbestos bodies

Most common manif of asbestos exposure | Well circumscribed plaque of dense collagen containing calcium on parietal pleura

Pleural plaque

Asbestosis begins

in lower lobes and subpleura

Asbestos exposure is linked with

``` 1 parenchymal intersitial fibrosis 2 plaques/diffuse pleural fibrosis 3 pleural effusion 4 lung cancer 5 malignant pleural mesothelioma 6 laryngeal cancer ```

Causes pneumonitis and interstitial fibrosis

Bleomycin

Causes pneumonitis and fibrosis

Amiodarone

1 month post radiotherapy Fever, dyspnea, pleural effusion, pulmo infiltrates Resolve with corticosteroid therapy progressing to chronic radiation pneumonitis

Radiation pneumonitis

Multisystem disease of unknown etiology characterized by noncaseating granuloma in organs Diagnosis of exclusion in young less than 40, nonsmoker Mc manifestation

Sarcoidosis Bilateral hilar lymphadenopathy or lung involvement

Sarcoidosis pathogenesis

Disordered immune regulation in genetically predisposed persons

Diagnostic histopath feature in sarcoidosis

Noncaseating epitheloid granuloma irrespective of organ involved

Laminated concretion of calcium and protein

Schaumann body

Stellate inclusion enclosed within giant cell

Asteroid bodies

Uveoparotid involvement in sarcoidosis

Mikulicz syndrome

CD4 cells secreting THI Independent cytokines IFN y IL2 LN enlargement, sicca syndrome, erythema nodosum, visceral invovlement Lung 90% with granuloma formation and intersitial fibrosis

Sarcoidosis

Immunologically mediated and delayed type hypersensitivity inflammatory lung disease primarily affecting alveoli Type III and IV (caseating granuloma) hypersensitivity

Hypersensitivity pneumonitis

Mold’s hay exposure | Micropolyspora faeni

Farmer’s lung

Moldy sugar cane Bagasse Thermophilic actinomycetes

Bagossis

Moldy maple bark | Cryptostroma corticale

Maple bark disease

Cool mist humidifier Actinomycetes Aureobasidium pullulans

Humidifier lung

Mold barley | Apergillus clavatus

Malt worker’s lung

Moldy cheese | Penicillium casei

Cheesewasher’s lung

``` Dust contaminated grain Sitophilus granarius (wheat weevil) ```

Miller’s lung

Pigeon dropping | Pigeon serum protein in dropping

Pigeon breeder’s lung

Chemical industry | Trimellitic anhydride isocyanate

Chemical worker’s lung

Fever, dyspnea, hypoxia and diffuse pulmo infiltrate BAL 25% eosinophil Prompt response to corticosteroid

Acute eosinophilic pneumonia with respiratory failure

Transient pulmonary lesions eosinophilia Thickened alveolar septa with eo and giant cell

Simple pulmonary eosinophilia | Loeffler

Clinically silent emboli percentage Recurrence rate of PE

60-80% 30%

Hemoptysis Anemia Diffuse pulmonary infiltrate

Diffuse alveolar hemorrhage syndrome

Proliferative rapidly progressive glomerulonephritis Hemorrhagic interstitial pneumonitis Antibody against alpha3 of collagen IV

Goodpasture syndrome

diffuse alveolar hemorrhage hemosiderin linear immuneglobulin deposition in renal biopsy

Diffuse alveolar hemorrhage syndrome

Pulmo manifestation like Goodpasture but no renal disease or antibasement membrane antibody

Idiopathic pulmonary hemosiderosis

Upper respiratory or pulmonary manifestation Necrotizing vasculitis Parenchymal necrotizing granulomatous inflammation sinusitis, epistaxis, nasal perforation cough, hemoptysis, chest pain PR3 ANCA +

Wegener’s granulomatosis

Pneumococcal infections occur with inc frequency in

1 chronic disease (DM, COPD, CHF) 2 congenital or acquired IgG defect (AIDS) 3 sickle cell or splenectomy bec harbors phagocyte and produces antibody

Pneumococcal pneumonia is acquired by with lobes more involved

aspiration of pharyngeal flora 20% lower lobes and right middle lobe

4 stages of pneumonia

Congestion Red hepatization Gray hepatization Resolution

Heavy, boggy red, vascular congested lobe | Proteinacious fluid, scattered neutrophil and bacteria in alveoli

Congestion

Liver like consistency and alveolar spaces are packed with neutrophil, red cell, and fibrin

Red hepatization

Dry, gray firm | Lysed red cell with fibrinosuppurative exudate persists within alveoli

Gray hepatization

Uncomplicated case | Digestion of exudate to produce granular semifluid debris resorbed ingested by mac coughed up by fibroblast

Resolution

Complications of pneumonia esp sero type 3

1 abscess 2 empyema 3 organization of intraalveolar exudate converting lung to solid fibrous tissue 4 meningitis, arthritis, infective endocarditis

Gram + lancet shaped diplococci | Most common cause of CAP and a lobar inflammation

S pneumoniae

Most common bacterial cause of acute exacerbation of COPD

H influenzae

Causes epiglottitis and suppurative meningitis in children

H influenzae type B | Encapsulated

Inc cause of bacteria in elderly 2nd most common cause of acute exac of COPD in adult Causes otitis media along with H influenzae and S pneumoniae

M catarrhalis

Important cause of secondary bacterial pneumoniae in children and healthy adults after viral respi illness (measles, influenza) Assoc with abscess and empyema Also a cause of nosocomial p

Staphylococcus aureus

Staph pneumoniae with r sided staph endocarditis is a serious complication of

iv drug abuse

Most freq cause of gram neg bacterial pneumonia

Klebsiella pneumoniae

Pneumonia affecting alcoholics, debilitated and malnourished

Klebsiella

Thick gelatinous sputum is characteristic of Kleb bec

viscid capsular polysaccharide preventing coughing up

Nosocomial and cystic fibrosis related pneumonia Neutropenic, burns, mech vent Propensity to invade bv with extrapulmo spread Fulminant, death with coag necrosis of pulmo parenchyma invading walls of necrotic blood vessel

Pseudomonas aeruginosa Pseudomonas vasculitis

Pontiac fever(URTI) Water cooling towers, tubing system Inhaled aerosolized organism or aspiration of contaminated water Common in cardiac, renal, hema and organ transplant patient

Legionella pneumophila

L pneumonia may be dx by

L antigen in urine or + fluorescent antibody on sputum Standard: culture

Mosy common atypical pneumonia occuring in closed community

Mycoplasma pneumoniae

Red blue congested subcrepitant area Inflammatory reaction largely confined within walls of alveoli Widened edematous septa Alveolar space free of cellular exudate

Atypical pneumoniae

``` Moderate amount of sputum Absence of consolidation Moderate elevation of white cell Lack of alveolar exudate Respi distress out of proportion to clinical and radio signs ```

Atypical pneumoniae

Influenza subtypes are determined by

Hemagglutinin | Neuraminidase

Epidemics | Mutations of HA and NA antigen allowing them to escape most host antibodies

Antigenic drift

Pandemics HA and NA replaced through RNA recombination with that of animal viruses making all animals susceptible to new influenza virus

antigenic shift

Pulmo abscess resulting from aspiration of infective material are more common on

the right side with more vertical airway posterior segment of upper lobe apical segment of lower lobe

Lung abscess may rupture into

Pneumothorax | Empyema

Most common cause of death from single infectious agent

Single most important risk factor for development of TB

HIV

Polymorphism of this gene predisposes diseasr progression without development of effective immune TB response

NRAMPI

Immunity to TB is mediated by

THI cells stimulating mac to kill bacteria comes at cost of hypersensitivity

Most common opportunistic viral pathogen in AIDS

CMV