CHEST PATH Flashcards
name CF manifestations in different systems
Sweat gland
- decreased absoprtion of NaCl from lumen, thus high in sweat (salty sweat)
Lung
- Infection (Pseudomonas)
- ABPA (10%)
- Bronchial artery hypertrophy -> Hemoptysis
Pancreass
- Pancreatic insufficiency
- Chronic pancreatitis
- Atrophy of exocrine pancreas (from mucous plugging)
Intestine
- Meconium ileus
- Steatorrhea
Salivary glands
- Ductal dilatation
Liver
- Secondary biliary cirrhosis (cannaliculi plugging)
Testes
- azoospermia and infertility
- Absent vas deferens
Lung LCH pathophysio ?
- Immature dendritic cells
- S100 positive
- Birbeck granules
How many % of lung LCH are smokers ?
95%
M = F
Lung LCH associations
- ALL
- AML
Radiology findings of LCH
- Upper zone Centrilobular cavitating nodules (early)
- Spares costophrenic and costomediastinal angles
- Normal lung parenchyma
- Bizarre shaped cysts, thin walled
- Fibrosis and honey combing (late)
Lung LCH prognosis
- May regress if stop smoking
- End stage pulmonary fibrosis if continue smoking
Lung LCH complications
- Pneumothorax (first presentation)
- Pneumomediastinum
- Interstitial fibrosis
- Pulmonary HTN and Cor pulmonale
Lung LAM inheritance ?
- Spontaneous (most common)
- TSC1 and TSC2
Lung LAM pathophysio ?
- Proliferation of smooth muscle cells* in lung
Extra-thoracic findings of LAM
- renal AMLs (even if no TS)
- Chylous ascites
- Lymphangioleiomyomas
- Uterine leiomyomas
Prognosis and treatment of LAM
- slow progressive over decades
- lung transplant only definitive treatment
LAM complications
- Pneumothorax (recurrent, bilateral)
- AML bleed (often fatal)
- Hemoptysis
- Lung infections
- SEGA obstructing (death)
- Osteoporosis (in immobile pts)
LIP epidemiology
- F (because Sjogren syndrome most common in F)
- middle age adults (most common)
- child (have AIDS)
LIP associations
- Sjogren syndrome (most common - 25%)
- AIDS (children)
- Connective tissue diseases
- SLE
- Castleman
- RA
- Pulmonary amyloidosis
Radiological features of LIP
- mid to lower zone
- small centrilobular nodules
- ground glass changes
- thin walled cysts
- mediastinal lymphadenopathy
LIP complication
- Develop into Lymphoma* (5%)
- More than 30% of patients will develop end-stage disease and honeycombing despite treatment.
UIP / IPF Risk factors
- Smoking (most important, increases several folds)
- Metal fumes
- Wood dust
- Farming, Hair dressing, Stone-polishing
- GORD*
Microscopic of UIP
- **Spatial and temporal heterogeneity (fibrotic lesions at different stage)
- Patchy interstitial fibrosis is hallmark
- Fibroblastic foci (early stage)
UIP complications
- Pulmonary HTN
- Cor pulmonale
- Lung cancer
- Pneumothorax/Pneumomediastinum
- Pneumonia
UIP Aetiologies
Connective tissue disorders
- RA
- Scleroderma
- Polymyositis/Dermatomyositis (UIP, NSIP, COP)
- Asbestosis
- Hypersensitivity Pneumonitis
- Amiodarone
- ANCA vasculitis
- Radiation
UIP epidemiology
- M > F (NSIP Female)
- Older patients (NSIP younger)
NSIP epidemiology
- F > M (due to high association to collagen vascular disease)
- 40-50yo
NSIP associations
NOT associated with smoking
Connective tissue disorders
- Scleroderma
- SLE
- Sjogrens
- Polymyositis
- Dermatomyositis
- IgG4
Autoimmune
- RA
- PBC
- Hashimoto thyroiditis
- Hypersensitivity pneumonitis
- Chemotherapy: Thalidomide
Microscopic of NSIP
- Temporal and spatial homogeneity (c.f. UIP is Heterogeneity)
- NO acute lung injury
- NO hyaline membranes
- NO granulomas
- NO organisms or viral inclusions
- NO dominant airways disease or organising pneumonia, eosinophils and coarse fibrosis.
Treatment and Prognosis of NSIP
Prognosis
- Overall better prognosis 45% full recovery
Treatment
- Steroids effective*
Patho appearance of COP
- Alveolar and bronchiole inflammation
- Polypoid plugs of loose organising connective tissue (Masson bodies)**
- The connective tissue is of the same age**
- Underlying lung architecture is normal. No interstitial fibrosis or honeycomb lung**
COP Complications and Prognosis and Treatment
Prognosis: Waxing and waning course, often treated for months for “recurrent” pneumonia.
Treatment: Some recover spontaneously, most need oral steroids for 6 months + for full recovery.
ABPA microsscopic findings
- Charcot Leyden crystals (seen in asthma and eosinophilic diseases)
- Bronchocentric granulomatosis
- TH2 helper cell response
Asbestos 2 types ?
Serpentine Chrysotile - White: Least pathogenic
Amphiboles - Blue: Includes crocidolite (most pathogenic)
do lung carcinoid tend to metastasize ?
- no
how many % get carcinoid syndrome ?
10% get carcinoid syndrome
Survival rate for lung carcinoid
5 year survival 95%
Cardiac myxoma site?
- Most common cardiac tumor
- 90% of myxomas arise in the atria, with a L to R ratio of 4:1
- Usually single
- Region of the fossa ovalis in the atrial septum is the favoured site of origin.
Cardiac myxoma
Carney complex
Dilated cardiomyopathy causes
- Idiopathic (most common)
- Genetic, alcohol, peripartum, post-viral, post-MI, haemochromatosis, sarcoidosis
Hypertrophic Cardiomyopathy causes
- 100% genetic
- sarcomeric proteins defect
Restrictive cardiomyopathy causes
o Idiopathic
o Amyloidosis
o Radiation
o Infiltration (amyloidosis, sarcoidosis, haemochromatosis, metastases), eosinophilic
endomyocardial disease (Loffler), endomyocardial fibrosis (tropical areas)
Cystic fibrosis mutation
- CF transmembrane conductance regulator
(CFTR) gene on chromosome 7. - The most common specific mutation is “delta F508 mutation”.
What renders a lung cancer unresectable ?
T4: Invasion of mediastinal structures, vertebral body, tumor nodule(s) in separate ipsilateral lung lobe
N3: Metastases to scalene, supraclavicular, contralateral mediastinal, or contralateral nodes
M1: Metastatic disease (malignant pleural effusion)