▪ Characteristics: Whole or part of lung is collapsed or suffers without air.

▪ Pathogenesis: Loss of lung vol due to alveolar collapse → ↓ oxygenation → V/Q mismatch

▪ Types

1. Resorption

2. Compression

3. Contraction

4. Neonatal
▪ Cause: complete airway obstruction in bronchi, subsegmental bronchi or

bronchioles due to mucus/mucopurulent plug following surgery/ aspiration

of foreign material (mostly R. side of lungs) /bronchial asthma, chronic
bronchitis, bronchiectasis, bronchial neoplasms

▪ Pathogenesis: Prevents air from reaching alveoli → resorption of air trapped

in distal airspaces through the pores of kohn (gives collateral ventilation) →

lack of air in distal space → collapse

▪ Symptoms: Fever and dyspnea within 24-36 hrs of collapse + IPSILATERAL

deviation of trachea and diaphragmatic elevation + absent breath sounds

and absent vocal vibratory sensation + Lack of lung expansion on
inspiration
▪ Cause: Tension pneumothorax/pleural effusion

▪ Pathogenesis: Air/fluid accumulation → increased P →

collapsed underlying lung

▪ Symptoms: Trachea and mediastinum shift AWAY from

atelectatic lung
▪ Causes: Local/diffuse fibrosis affecting the lung or the pleura hamper

lung expansion

▪ Irreversible when its due to fibrosis/ but reversible if not due to

fibrosis

▪ Treatment: treat promptly to prevent hypoxemia and superimposed

infection of collapsed lung.

▪ Complications: V/Q mismatch, Hypoxemia

▪ Normally surfactant is a lipoprotein which is synthesized ~ 28 weeks of gestation by Type II pneumocytes

and stored in lamellar bodies. Protein A/D provide innate immunity while protein B/C reduces surface
tension at air liquid barrier in alveoli → prevents collapse on expiration. Most of it is synthesized by ↑
cortisol and thyroxine + ↓ insulin

▪ Cause: RDS in newborns due to premature delivery, Maternal diabetes (Fetal Hyperglycemia stimulates

Insulin release), C-section (Lack of stress from vaginal delivery → less cortisol)

▪ Symptoms: respiratory distress within few hours of birth, hypoxemia, respiratory distress, ground glass

appearance on chest X-ray (all 3 must be present, only positive histo slide is NOT enough)

▪ Complications: Intra-ventricular (lateral ventricles) hemorrhage in brain, PDA (persistent hypoxemia),

Necrotizing enterocolitis (interstitial ischemia), Hypoglycemia (increased insulin release), O2 therapy –

Reperfusion Damage to lungs (bronchopulmonary dysplasia) and cataracts (Blindness)
Histo: collapsed alveoli are lined by hyaline
membranes
Airway disorder – trachea to terminal bronchiole, Increased resistance to airflow and limited
expiratory rates on forced expiration. Obstruction when air is trying to come out!
Reduced FEV1: FVC ratio, All lung capacity goes up due to air trapping (TLC, RV)!
▪ Epidemiology: M=F, 4th leading cause of death in USA
▪ Cause: Obstruction secondary to limitation of airflow due to Emphysema + chronic bronchitis
mostly due to SMOKING!
▪ Lab testing: ↑ TLC/RV , ↓ FVC, ↓↓ FEV1, ↓ FEV1: FVC ratio
▪ Characteristics: Abnormal PERMENANT airspace enlargement DISTAL to terminal bronchioles,
Accompanied by destruction of airspace walls, NO FIBROSIS
▪ Types:
▪ Centri-lobular - Respiratory bronchioles are affected and distal alveoli are spared,
▪ most common type due to smoking found in upper lung zones
▪ Diseased + normal air spaces in same lobule
▪ Pan-lobular – respiratory bronchioles TO terminal alveoli affected
▪ Due to α1 anti-trypsin deficiency – Normal function to inhibit neutrophil proteases
▪ Due to point mutation on chromosome 14 (PiMM → PiZZ), Abnormal protein does not get secreted into
serum, thus accumulates in liver → chronic liver disease
▪ Other mutations causing the diseases (polymorphisms in TGFβ and MMP)
▪ Found in lower lung zones
▪ Para-septal – Next to atelectasis, along septa margins of lobes, subpleural, most commonly found in
upper lobes → bullae formed and if rupture → pneumothorax
▪ Irregular – Surrounding scar but asymptomatic
▪ Imbalance in protease-anti-protease
mechanism (too much protease and
too LITTLE anti-protease),
▪ Normally protease comes from WBC
(neutrophils) to destroy lung tissue
and anti-protease inhibit these to play
a protective role.
Gross: Hyperinflated lungs with/without bullae formation, with parenchyma giving Moth-eaten
appearance

Histology: Large alveolar spaces with destruction of alveolar septa without fibrosis: Free floating
alveolar septa in air spaces. Destruction of elastin within small airways → airway collapse during
expiration → functional obstruction
▪ Symptoms: Dyspnea (insidious in onset and progressive), productive cough (depends on extent of
chronic bronchitis), weight loss (Hyperventilating and excessive puffing and panting known as Pink
puffers),
▪ Signs: Barrel chest (increased A-P diameter), prolonged expiration and pursed lips, sitting in
forward hunched position trying to squeeze air out of lungs
▪ Spirometry: ↑ lung volumes (TLC, RV), Decreased FEV1: FVC, ↓ DLCO (loss of surface area for O2)
▪ Chest x-ray: ↑ air trapping, flat domes of diaphragm
▪ Blood gas: Normal at 1st (pink puffers), later (Hypoxia, hypercapnia, respiratory acidosis)
▪ Characteristics: Persistent cough productive of mucoid sputum, for > 3
consecutive months in a year, for > 2 consecutive years, with NO OTHER
IDENTIFIABLE CAUSE
▪ Cause: most common amongst smokers and urban dwellers (inhaled
irritants: cigarette smoke, SO2, NO2)
▪ Pathogenesis:
▪ submucosal gland hypertrophy + goblet cell metaplasia in
bronchioles→ hypersecretion of mucus
▪ Smooth M. hyperplasia and peribronchiolar fibrosis → small airway
obstruction distally,
▪ Inflammation due to infiltration of CD8+ T-cells, Macrophages, and
neutrophils (NO EOSINOPHILS)
▪ Gross: Hyperemia + edema of mucus membranes + excessive mucinous
or mucopurulent secretions
▪ Microscopy: thickening of mucus gland layer (↑ Reid index > 0.4),
goblet cell metaplasia, Smooth M. hyperplasia, Peribronchiolar fibrosis,
squamous metaplasia (due to irritant)
▪ NEVER DO A BIOPSY
▪ Symptoms: Persistent cough productive of mucoid sputum overtime progresses to dyspnea on
exertion, cyanosis (Blue bloaters)
▪ Signs: Hypoxia, Hypercapnea, Cyanosis, persistent hypercapnia → respiratory centres
insensitivity to PCO2 so respiratory drive is driven by low PO2 (careful when giving O2 as it
diminishes the respiratory drive)
▪ Complication: Infections, overtime can lead to pulmonary HTN and cor-pulmonale (RHF due to
lung issue)
▪ Characteristics: Chronic inflammation disease of airways, characterized by
bronchial hyperactivity,
▪ Symptoms: EPISODIC (mostly at night/early mornings), REVERSIBLE
bronchoconstriction, Recurrent wheezing, breathlessness, chest tightness
and cough, Increased mucus secretions (thick sputum), hyper-inflation of
lungs with air trapped distal to mucus packed bronchi, tachypnea
▪ Spirometry: ↓ FEV1: FVC, ABG (hypoxia, hypercapnia, respiratory acidosis)
▪ Complications: status asthmaticus (severe prolonged unresponsive to
therapy) – need emergency treatment
▪ Common triggers: Drug induced, occupational, seasonal, exercise induced
▪ Gross morphology: Occlusion of airways with thick mucus, hyperinflation of
lungs
▪ Histology:
▪ Edema, patchy epithelial necrosis
▪ Bronchial lumen: Mucus plugs with curschmann spirals (whorls of shed
epithelium), charcoat leyden cyrstals (eosinophil crystalloids) , inflammation
WITH eosinophils, lots of goblet cells, bronchial smooth M hypertrophy and
hyperplasia, BM thickening
▪ Atopic type: Increased IgE
▪ 1st exposure inhaled allergen elicits Th2 response in genetically predisposed patients → cytokine
release (IL4 stimulates IgE production (class switch), IL5 activates eosinophils, IL 13 stimulates
mucus production and promotes IgE production by B-cells) → IgE coats mast cells which
degranulate with Ag exposure
▪ Further exposure:
▪ Early phase: within minutes causes bronchoconstriction (vagal stimulation), increased mucus
production, vasodilation, increased permeability, further recruitment of leukocytes
▪ Late phase: 4-24 hrs activates eosinophils, PMNs, T cells, epithelial cells are activated and recruit
more Th2 cells and eosinophils
▪ Non-atopic type: Normal IgE, and no evidence to sensitization
▪ 2° to viral infection of URT/inhaled SO2, NO2, O3 causes mucosal damage → lower threshold of
subepithelial vagal receptors to irritants → ultimate inflammation (same as atopic type)
▪ Drug induced: Aspirin mediated reaction causing rhinitis, nasal polyps, urticaria and bronchospasm
▪ Inhibit COX → more shunting towards lipo-oxygenase pathway → more leukotrienes → more
bronchoconstriction
▪ Occupational: pt goes to work → asthma, comes home fine..
▪ Stimulated by epoxy, resins, plastics, wood, cotton, platinum, toluene, following repeat exposure
▪ Characteristics: Permanent dilation of brochi/bronchioles PROXIMAL to air spaces +
Destruction of supporting tissue + Associated with CHRONIC NECROTIZING
INFECTION
▪ Cause: Obstruction (tumor, foreign body), congenital conditions (Kartagener, CF),
necrotiziing/suppurative pneumonia (virulent organisms S. aureus, Kleibsiella)
▪ Pathogenesis: obstruction prevents clearance → pooling of secretions behind the
obstruction → 2°infection → damaged/weakened bronchial walls → bronchiectasis
▪ Gross finding: Bronchioles extending till the edge of the lungs/pleural surfaces,
dilated bronchi appear as cysts filled with mucopurulent secretions
▪ Histo: Acute + chronic inflammation in bronchial walls, necrotizing ulceration,
squamous metaplasia of bronchial epithelium, Lung abscesses, fibrosis of bronchial
walls, if fully filled (bronchiolitis obliterans)
▪ Symptoms: Severe persistent productive cough which is extremely FOUL smelling
sputum (due to infection), dyspnea, hemoptysis, Clubbing (Rule out cancer),
cyanosis
▪ Characteristics: AR disorder of ciliary microtubules (structural abnormality of cilia
impaired mucociliary escalator)
▪ Complications: Persistent infection → bronchiectasis, Decreased spermatozoa motility
→ sterility in males
▪ Kartangener’s syndrome – Bronchectasis + sinusitis + situs inversus (all organs on
opposite site)
Parenchymal disorder – Respiratory bronchioles, alveoli and alveolar ducts, decreased expansion
with reduced total lung capacity, O2 diffusing capacity, lung volumes and compliance
▪ Characteristics: Diseases affecting lung parenchyma/interstitium (BM of
endothelial/epithelial cells filled with collagen, elastin, fibroblasts,
inflammatory cells) causing fibrosis + inflammation
▪ Pathogenesis: some damage to pneumocytes + endothelial cells (Alveolitis) →
leukocytes release cytokines which mediate and stimulate interstitial fibrosis
→ ↓ lung compliance → ↓ lung expansion during inspiration
▪ Symptoms:
▪ FIBROSIS → ↓ lung compliance → increased work to breath → dyspnea
▪ Interstitium damage → V/Q mismatch → hypoxia → tachypnea + Cyanosis

▪ Spirometry: ↓ lung volume due to ↓ flow rates → normal/↑ FEV1: FVC, ↓

DLCO
▪ Chest X-ray: Bi-lateral infiltration (reticulo-nodular/ infiltrative (Ground-glass
appearance)
▪ Complications: Progression to respiratory failure with pulmonary HTN + Cor
pulmonale
▪ Cause: Direct (Pneumonia, Aspiration, emboli, inhalation injury, drowning, O2 toxicity), Indirect (sepsis,

Trauma with shock, Acute pancreatitis, severe burns, transfusion products, Uremia, drug reactions)

▪ Symptoms: Rapid onset (within a week), Severe hypoxemia, Bilateral pulmonary infiltrates (X-ray),

refractory to O2 Therapy, 2° to direct/indirect lung injury

▪ Prognosis: depends on heritable and non-heritable conditions, High mortality rate (40%), Supportive

therapy, Poor prognosis if (advanced age, bacteremia/sepsis, Progression to multi-system organ failure),
return to normal function after 6-12 months (some part of lung is spared mostly if survived during acute
state, part that has undergone fibrosis is NOT reversible. )

▪ Chemical mediators – cytokines, Oxidants, growth factors – TNF, IL-6, IL-10, TGF-β
Alveolar capillary
membrane compromise
due to Endothelial or
epithelial injury→ ↑
vascular permeability →
loss of diffusion and
deficiency of surfactant if
Type II cells are damaged.
▪ Acute phase – 0-4 days

▪ Gross: Heavy and firm lungs , Interstitial and intra- Acute phase: Some alveoli are collapsed, while
others are distended; many are lined by bright
alveolar edema/hemorrhage, Necrosis and sloughing of
pink hyaline membranes (arrow).
alveolar epithelial cells

▪ Histology: Hyaline membranes, Diffuse alveolar damage

▪ Organizing phase: 4 days – 3 weeks

▪ Gross: Alveolar septal thickening

▪ Histo: Proliferation of Type II cells, organization of fibrin

exudates → fibrosis, Resorption of hyaline membrane

Organizing phase:. reactive type II
pneumocytes also are seen at this stage
(arrows),
▪ Epidemiology: M > F, 2/3 pts > 60 yrs
▪ Characteristics: Progressive bi-lateral interstitial fibrosis → severe hypoxia + cyanosis, radiologic
diagnosis (Usual interstitial pneumonia)
▪ Prognosis: Poor 5 year survival ~ 20%, mean survival < 3 yrs
▪ Symptoms: Dry cough + Dyspnea on exertion (Progressive), crackles on inspiration, cyanosis
▪ Complications: Cor-pulmonale, clubbing
▪ X-ray: ↑ interstitial markings in lower lobes, Honeycombing (cyst-like spaces due to fibrosis)
▪ Gross finding: Cobblestone pleural space, firm, fibrotic parenchyma with lower lobe and sub-pleural
accentuation, honeycomb cysts
▪ Histo: Temporal (mature/immature fibrosis together), geographical heterogeneity (only some areas
affected), Honeycomb change – cystic space lined by Type II pneumocytes/respiratory epithelium
(dense fibrosis will destroy the architecture of parenchyma creating cysts)
▪ Treatment: Lung transplant
▪ Epidemiology: Younger children
▪ Treatment: Steroids, Immunomodulator therapy
▪ Histology: Uniform fibrosing process (chicken-wire appearance), NO
HONEYCOMBING, FIBROBLAST FOCI
▪ Types: Cellular (lymphocyte infiltrate – better), Fibrosing type (worst)
▪ Cause: UNKNOWN
▪ Symptoms: cough, dyspnea
▪ X-ray + Histo: Patchy, peri-bronchial, subpleural consolidation
▪ Mostly associated with Lupus, rheumatoid arthritis, systemic sclerosis,
dermatomyositis-polymyositis
▪ Pleural involvement can occur with pleuritis, pleural nodules, pleural effusion
▪ Non-neoplastic lung disease 2° to inhalation of mineral dust causing
▪ Pathogenesis: Dust induced fibrosis (particles inhaled → taken by macrophages → release of
lysosomal enzymes and free radicals while phagocytosis takes place → inflammation causing tissue
injury + secretion IL-8, IL-1 which leads to fibroblast proliferation → fibrosis
▪ Types
1. Silicosis – fibrogenic
2. Asbestos - fibrogenic
3. Coal workers pneumoconiosis – weakly fibrogenic
▪ Cause: Inhalation of silica mostly from mining,
demolition, stonecutting, sandblasting, grinding,
foundry work, ceramics
▪ Complication: Pulmonary TB ( silica inhibits the ability
of pulmonary macrophages to kill phagocytosed
myobacteria)
▪ Gross: collagenous nodule/scar (hilar lymph nodes/
upper lung fields), progressive massive fibrosis
▪ CXR: Eggshell calcification (sheets of calcifications in
lymph nodes)
▪ Histo: central area of whorled collagen fibers with dust-
laden macrophages, eosinophilic substance, weakly
birefringent under polarized light
▪ Symptoms: SOB (first at exertion then at rest), slow
progression generally,
▪ Cause: Due to inhalation of asbestos fibers due to exposure
to mining, milling, insulation, construction, demolition.
▪ Complication: Tumor initiator + tumor promoter due to free
radical injury → lung carcinoma + mesothelioma (chest pain
present), cor pulmonale, respiratory failure
▪ Histo: asbestosis body – golden brown, beaded rods with
translucent center covered by iron-containing proteinaceous
material (from macrophages)
▪ Gross: Peribronchiolar fibrosis → diffuse interstitial fibrosis,
pleural Plaques (over domes of diaphragm, made up of
dense collagen and calcification but no asbestos bodies seen
in pleura), mostly in lower lung lobes
▪ Symptoms: Diffuse interstitial lung disease, dyspnea, cough
▪ ASBESTOS + SMOKING = You’re screwed
▪ Cause: Inhalation of coal dust (coat dust ingredients: carbon, silica and

other minerals, metallic and organic compounds)

▪ Gross: in sequence

1. Anthracosis: accumulation of carbon pigment mostly in peri-lymphatic

regions and lymph nodes (asymptomatic as no fibrosis)

2. Simple CWP: macules + nodules ( aggregates of dust-laden

macrophages, minimal/absent fibrosis), little/no pulmonary

dysfunction

3. Complicated CWP: coalescence of nodules into fibrous scars,

impaired pulmonary function, only 10% progress till here

▪ Characteristics: Non-necrotizing epithelioid granulomata in many tissues and organs
▪ Epidemiology: Young adults < 40 yrs, Danish, Swedish, African American population, More in non-
smokers (F >M)
▪ Pathogenesis: Cell mediated (Type IV hypersensitivity) reaction to Ag.
▪ CD-4 accumulate in intra-alveolar and interstitial space → secrete IFN y + IL-2 → Peripheral depletion
→ No reaction to skin test with candida (unable to mount immune response – Anergy)
▪ Association: Polyclonal hyper-gammglobulinemia
▪ Complication: Leads to End-stage lung disease
▪ Symptoms: asymptomatic, gradual respiratory symptoms, Erythema nodosum (skin nodules), sicca/sjoren
syndrome (eyes/lacrimal gland involved, dry eyes, dry mouth and CT disorder)
▪ Labs: hypercalcemia (activation of vit D by epitheloid cells), high ACE
▪ CXR: Bilateral hilar lymphadenopathy, fibrosis
▪ Histo: Non-necrotizing granuloma in lymphatic distribution, collagen → granuloma overtime, schaumann
bodies (laminated concretions of Ca and protein), Asteroid bodies (stellate inclusion in giant cells)
▪ Prognosis: recurrence in transplant pts
▪ RULE OUT EVERYTHING especially infection before diagnosis
▪ Cause: Inhaled Ag causing granulomatous interstitial pneumonitis
→ restrictive lung disease
▪ Farmer’s – Moldy hay, thermophilic bacteria (saccaropolyspora
rectivirgula)
▪ Silo’s filler’s disease: inhalation of gases from plant material (oxides of
nitrogen)
▪ Byssinosis: cotton, linen, hemp, textile factory workers, Monday
morning blues
▪ Pathogenesis:
▪ Type III hypersensitivity (1st exposure – Ig in serum, 2nd exposure – Ab
combine with inhaled Ag to form immune complexes → inflammatory
response in lung)
▪ Type IV (chronic exposure)

▪ Histo: Airway centred process, chronic inflammatory infiltrate,

organizing pneumonia, poorly formed non-granulomas with giant
cells
▪ Respiratory bronchiolitis associated interstitial lung
disease – Macrophages accumulate WITHIN the lumen
of respiratory bronchioles associated with
peribronchiolar fibrosis, and responds to steroids and
smoking cessation
▪ Desquamative interstitial pneumonia - fills alveolar
spaces by macrophages, minimal interstitial fibrosis
▪ Pulmonary Langerhans cell histiocytosis – Eosinophilic
granuloma but no significant fibrosis
▪ Aggressive form of interstitial lung disease
▪ Symptoms: Present like ARDS
▪ Characteristics: Diffuse alveolar damage + Hyaline membranes
▪ Associated conditions: Can occur with IPF (Interstitial pulmonary fibrosis) as the acute phase
▪ Cause: Mostly due to infectious pneumonia, transplantation (lung and bone marrow),
Collagen vascular disease
▪ Treatment: spontaneous/ with steroids
▪ NO known cause
▪ Characteristics: Expansion of interstitium by groups and sheets of lymphoid cells
▪ Associated conditions: HIV, CT disease, auto-immune
▪ Complication: Lymphoma
▪ Symptoms: Presents like ARDS
▪ Characteristics: accumulation of fluid into
alveolar spaces → decreased diffusing
capacity, hypoxemia, SOB
▪ Cause:
▪ Cardiogenic – due to LHF (check for high PCWP)
▪ Non-cardiogenic – ARDS, high altitude, PE,
neurogenic (head trauma, Hemorrhages)
▪ Symptoms: SOB, frothy sputum, inspiratory
crackles
▪ CXR: Bi-lateral haziness
▪ Characteristics: Blood clot travels to the lungs lodging within pulmonary artery or downstream branch
mostly originating from larger, deeper veins of legs/pelvis.
▪ Cause: air, fat (fracture) , amniotic fluid (post-partum), IV drugs, bone marrow (due to CPR)
▪ Epidemiology: COD in 50,000 pts/yr in USA
▪ Pathogenesis: Virchow’s triad (Endothelial injury, hypercoagulability, abnormal blood flow)
▪ Risk factors: Immobility (airplane, flight, bed rest), surgery (orthopedic), Severe trauma (burns –
endothelial injury, fractures), CHF, elevated estrogen – HRT/ Oral birth control pills (Hypercoagulibitlity),
adenocarcinomas (Trousseau syndrome (random thrombi in venous circulation)), Hypercoagulibility
syndromes ( factor V, Protein C/S, anti-thrombin III deficiency, lupus)
▪ Symptoms: depends on size, cardiopulmonary status of pt (small emboli – no effect, Medium – only
causes symptoms if some pathology already present in heart or lungs, large – everyone’s screwed)
▪ SOB (10-15% pts), Sudden death if > 60% vasculature is blocked), recurrent emboli → pulmonary HTN and cor-
pulmonale
▪ Increased Pulmonary Artery Pressure – Proximal to the occlusion → RHF (acute cor-pulmonale)
▪ Ischemia of pulmonary parenchyma – Distal to occlusion → compromise of lung (hemodynamic + Pulmonary)
▪ Infarction: Rare due to dual blood supply (if occurs – red/hemorrhagic)
▪ Atelectasis – reduced surfactant production (less type II pneumocytes)
▪ Patent foramen ovale due to ↓ O2
▪ Saddle embolus: Large embolus lodged into
main pulmonary artery bifurcation, causes
sudden death due to hypoxia and Acute RHF
▪ Characteristics: Mean pulmonary artery P > ¼ of systemic HTN, mostly in young females
▪ Classification
▪ Primary – sporadic/ familial (AD) – mostly in young women
▪ Mutation in BMPR type II (normally BMPR binds to TGF B to inhibit smooth M proliferation)
▪ fatigue, syncope, dyspnea on exertion, chest pain, cyanosis, death from RHF within 2-5 yrs
▪ Treatment: vasodilators, anti-thrombotic agents, lung transplant
▪ Secondary – ↓ cross sectional area of blood flow due to chronic obstructive/lung disease, recurrent
pulmonary emboli, heart disease (mitral stenosis, L → R shunt – all cause backflow)
▪ All above causes endothelial cell dysfunction → decreased vasodilatory agents + increased vasoconstrictive agents +
growth factor production
▪ Reflects symptoms with underlying disease, respiratory insufficiency and R. heart strain

▪ Histo:
▪ Main elastic arteries (atheromas)
▪ Medium sized A (intimal cell and smooth M proliferation → wall thickening), small arteries/arterioles
(thickening, medial hypertrophy, reduplication of IEL and EEL)
▪ plexiform lesions (multiple lumina within a small artery at a branch point from larger artery, more in primary
HTN)
▪ Characteristics: Triad ( Hemoptysis, Anemia (due to blood loss), diffuse pulmonary infiltrate –
blood/fluid in interstitium)
▪ Causes:
1. Goodpasture,
2. Wegner’s granulomatosis
3. Idiopathic hemosiderosis,
▪ Cause: Auto-immune (Ab against a3 chain of type IV collagen → linear
deposition of IgG along BM of kidney (glomerular)+ Lung (alveolar) → RPGN +
hemorrhagic interstitial pneumonitis)
▪ Gross: Heavy lungs and red-brown consolidation (fresh + old hemorrhage)
▪ Histo – alveolar hemorrhage, patchy necrosis of alveolar wall, intra-alveolar
hemosiderin (use Prussian blue stain), septal thickening and reactive Type II
cells (for repair),
▪ Labs: chest X-ray for consolidation, IF studies (look for linear deposits)
▪ symptoms: Lung - hemoptysis, anemia, pulmonary infiltrate + Renal – hematuria,
RBC casts, edema, uremia
▪ Treatment: plasmapheresis (remove Ab), immunosuppressive therapy (prevent
Ab formation), renal transplant
▪ Characteristics: Auto-immune with Ab against C-ANCA/PR3 ANCA, forming
granulomas in lungs and URT + vasculitis of small/medium sized vessels + RPGN
▪ Epidemiology: M > F, middle aged-older individual
▪ Symptoms: pneumonitis with nodules and cavitary lesions, chronic sinusitis, mucosal
ulcerations of nasopharynx + renal (hematuria, proteinuria), rashes, myalgia, fever,
articular involvement, neuritis
▪ Histo: Geographic necrosis (only some areas), neutrophilic micro-abcess, loosely
formed granulomas with central area of necrosis, + vasculitis (necrotizing capillaries)
▪ Prognosis: if untreated dead within 1 year
▪ Treatment: steroids, cyclophosphamide, Anti-TNF, Rituximab
▪ Cause: WHO THE HELL KNOWS (when its not goodpasture or wegner we will just
call it this)
▪ Epidemiology: children > adults
▪ Histo: similar to goodpasture but no Ab to BM or associated renal disease
▪ Treatment: steroids and immunosuppression
▪ Epidemiology: 1st most common COD related to death in industrialized countries, ↑incidence in
F (higher smoking rates and high susceptibility) and ↓ in M.
▪ Progression: 5 yr survival for all stage (16%), 5 yr survival if localized lung disease (45%)
▪ Cause:
▪ smoking (60X risk if you some 2 ppd for > 20 yrs), if stop smoking – decreased risk after 15 yrs, passive
smoking – 2X – NOT ALL SMOKERS DEVELOP CANCER (~11% do)
▪ Asbestos: (5X risk)
▪ Asbestos + Smoking = 55X risk

▪ Types and common mutations

▪ Non-small cell carcinoma – 3p del, P16, CDKN2a
▪ Signet ring adenocarcinoma, non-smokers: ALK

▪ Metastasis: Mostly lymph nodes but other areas: Adrenals (50%), liver, brain, bone
▪ if peripheral mostly silent, If central (obstruction, infection – pneumonia, abscess, bronchiectasis, atelectasis)
▪ Cough (mass irritates airway)
▪ Weight loss (cachexia),
▪ Hemoptysis ( mass erodes into vessels),
▪ Dyspnea, (pulmonary osteoarthropathy (clubbing),
▪ Hoarsness (recurrent laryngeal N,)
▪ Chest pain (pleural involvement, infiltration of tumor into chest wall),
▪ pericardial and pleural effusion → cardiac tamponade
▪ Persistent atelectasis, pneumonitis,
▪ SVC syndrome due to compression
▪ Horner’s syndrome (cervical sympathetic plexus damaged → ipsilateral ptosis, meiosis, anhidrosis, enophtalmos)
▪ Diaphragm paralysis
▪ Dysphagia
▪ Rib destruction
▪ Tumor at apex of the lung – mostly squamous cell carcinoma
▪ T1/T2 destruction → wasting of hand muscles, pain in arms (Ulnar N. involved)
▪ Horner – sympathetic ganglia blocked
▪ Edema (compression of BV)
▪ Recurrent laryngeal N paralysis (hoarsness)
▪ Dysphagia (esophagus involved)
▪ Thoracic duct obstruction (chylothorax – lymphatic fluid in pericardial cavity)
 Proliferation of atypical
▪ Progression: Atypical adenomatous hyperplasia
pneumocytes along septa
(precursor lesion, minimally atypical cells < 5 mm
diameter found adjacent to adenocarcinomas) →
Adenocarcinoma in situ (aka bronchioloalveolar
subtype, dysplastic cells growing along pre-existing
septae → apparent thickening of alveolar walls) →
minimally invasive adenocarcinomas with stromal
invasion (< 3cm total size and < 5mm invasive focus)
→ Invasive adenocarcinomas Adenocarcinoma in-situ
▪ Epidemiology: most common type of primary lung
tumor, F > M especially non-smokers, < 45 yrs
▪ Mutations: KRAS (poor prognosis), EFGR (good
prognosis because better response to medications)
▪ Characteristics: peripheral location, metastasis
widely at an early stage, 5 yr survival ~ 10%
▪ Histo: gland + mucin production
Adenocarcinoma with its
▪ Grading: if well formed glands (G1), barely any special marker
glands (G3)
▪ Associated with trousseau syndrome
▪ Characteristics: centrally located, mostly spreads to hilar lymph nodes,
fast growing tumors → central necrosis → cavitation, 5 yr survival ~
10%
▪ Epidemiology: M > W, Increased risk with smoking history
▪ Complications: can cause obstruction, atelectasis, infection
▪ Progression: squamous metaplasia → squamous dysplasia →
squamous cell in-situ → invasive squamous cell carcinoma
▪ Histo: Keratin pearls, intercellular bridges (connection between cells) ,
individual cell keratinization (keratin within cell → eosinophilic)
▪ Paraneoplastic syndrome: Hypercalcemia (pth like peptide)
▪ Grading: clear keratin pears (G1), no keratin pearls (G3)
▪ DO NOT GIVE bevacizumab → life threatening risk of
hemorrhage
▪ Diagnosis of exclusion – if nothing
lets call it a new type of cancer!!!
▪ Characteristics: poor prognosis as
early metastasis
▪ Paraneoplastic syndrome– Cushing’s (ACTH), SIADH,
myasthenic like syndrome, gastrin releasing peptide,
calcitonin, Lambert eaton
▪ Mutations: 3p deletions, myc amplification, p53, Rb
▪ Characteristics: centrally located, early metastasis to
mediastinal lymph nodes
▪ Epidemiology: 99% pts have smoking history
▪ Histo: round, scant cytoplasm (increased nuclear:
cytoplasm) , finely granular nucleus (Salt-pepper
chromatin), fragile cells → crush artifacts, nuclear
molding, extensive necrosis, frequent mitosis
▪ Treatment: good response to chemo
▪ 5 yr survival ~ 3%
▪ NSCLC pts with positive EFGR treatment respond better to TK inhibitors than pts
without mutation
▪ Adenocarcinomas + unspecified NSCLC pts more likely respond to pemetrexed
than squamous cell carcinoma
▪ Crizotinib is very useful in NSCLC as it inhibits EML4/ALK fusion protein (found on
45% pts)
▪ Characteristics: centrally located, polyploid growth, can metastasize to
lymph nodes
▪ Cause: Neuroendocrine tumor of lung arising from kulchitsky cells
▪ Epidemiology: ~ 40 yrs
▪ Symptoms: obstruction, cough, hemoptysis, infection
▪ Progression: diffuse pulmonary neuroendocrine cell hyperplasia →
carcinoid tumorlets → carcinoid tumor Typical → carcinoid tumor
atypical
▪ Treatment: resectable → good prognosis
▪ Complication: carcinoid syndrome (neuropeptides secreted into
systemic circulation → lots of serotonin → episodic attacks of flushing,
cyanosis, diarrhea, cramps, vomiting
▪ Histo: Nested appearance, uniform round cells, neuroendocrine
chromatin, no pleomorphisms, rare mitoses → no necrosis, salt/pepper
chromatin
▪ Characteristics: primary malignancy of pleura, peritoneum or
pericardium, Increased risk with asbestos
▪ Symptoms: chest pain, dyspnea (may/may not), cough, fatigue
▪ Imaging: moderate - large unilateral pleural effusion, nodular
thickening, Entire lung is involved epitheloid

▪ Histo: can mimic other types of malignancy (epitheloid/

sarcoid/ mixed type)
▪ Progressive: Progressive an fatal with 1-2 yr survival post
diagnosis
▪ Treatment: multi-modality (surgery + radiation + chemo)
Mixed
▪ Very common but usually incidental finding
▪ Characteristics: solitary 3-4 cm tumor in diameter,
appears rounded, radio-opacity on CXR
▪ Histo: nodules of CT (cartilage, fibrous tissue, fat) along
epithelial clefts