Respiratory 2

Question 1

What are restrictive lung diseases?

Answer 1

The amount of air the lungs are able to hold has decreased due to disease.

2 Types

• Interstitial
  (Lung tissue is damaged - so air has difficulty getting in due to lack of elascity by lung tissue)

ex -
0 Pneumoconiosis ( group of interstitial lung diseases caused by inhalation of certain dust particles which damage lungs ) - can be developed due to occupational, exposure.
* Coal workers Pneumoconiosis ( inhale coal particles or carbon dust)
* Silicosis (inhale silica particles - seen in silica miners & sand blasters )
* Asbestosis - ( seen in construction & ship yard workers )

0 Sacoidosis - form grannuloma

0 Hypersensitivity pneumonitis ( lungs develop an immune response to particles breathed in which results in inflammation of the lung & leads to fibrosis )
e.g. Farmer’s lung - reaction to moulds on straw etc.
Bird fancier’s lung - partciles from bird droppings

0 Idopathic Pulmonary fibrosis.

Overall in these disease processes —-> chronic inflammation occurs —–> cytokines & inflammatory mediators damage the lung , aswell as damage from partciles ———————-> extracellular matrix collagen deposited and replace healthy lung tissue causing fibrosis.
(SYMPTOMS WILL BE THE SAME AS PULMONARY FIBROSIS CARD)

• Extra - pulmonary - Structures surrounding lung are damaged —-> prevents chest expansion ( so air has difficulty getting in due to lack of space )

ex -
0 Obesity ( lot of fat around lungs)
0 Pleural effusion - fluid around lungs
0 Pectus exavatum - Breastbone ( sternum ) is sunken into chest ( can impair function of the heart & lungs ) / Congenital defomity of chest which causes ribs & breastbone to grow inwards.

0 myasthenia gravis ( affects the ability of the diaphragm - so lungs not able to expand )

IDIOPATHIC RESTRICTIVE LUNG DISEASE - IF NO REASON FOR RESTRICTIVE LUNG DISEASE CAN BE FOUND

Question 2

How does restrictive lung disease affect PFTs ?

Answer 2

Reduced :
Total lung capacity - the total amount of air the lungs can hold.
FEV1 - The amount of air that is echaled within 1 second.
FVC - Forced Vital capicity - the amount of air that can be forcibly breathed out after taking the deepest inhalation of breath possible.

( but normal or high FEV1 /FVC ratio as both have decreased , but also FVC is reduced more bcc fibrotic lung still has elastic recoil and allows air is be pushed out faster during expiration ?)

Question 3

What is Hypersensitivity pneumonitis ?

Answer 3

HISTORY TAKING IS IMPORTANT (as has similar symptoms to other lung conditions)

A group of condition where there is
Inflammation of alveoli & distal bronchioles due immune response against inhaled allergen e.g bacteria or non - human protein (plant or animal e.g same ones than cause farmer’s lung , bird fanciers disease)

Examples :
- Bird fanciers 
- 
- Farmer's lung
- malt worker's lung 
- wine marker's lung 
etc( if working in something do with manufacturing or growing - could be the cause) 

SUBTYPES
0 non-fibrotic hypersensitivity pneumonitis:
purely inflammatory

0 fibrotic hypersensitivity pneumonitis: mixed inflammatory/fibrotic or purely fibrotic

HOW DOES IT PROGRESS.- no longer really used bcc of difficulty differientiating btw stages.

( Acute - Inflammation is at its most intense - Fever, dyspnoea , tachypnoea , pulomary infiltrates (substance denser than air - blood , pus . protein ) , restrictive PFTs , reduced disffusing lung capcity of CO (DLCO) due to lymphocytic alveolitis ———————————————————————————->2. Sub acute ( inflammation reduces in intensity —->Fibrosis is increasing ( insidous development - gradually developing , subtle but very harmful )——> malaise , dyspnae , misxed picture PFT and reduced DLCO ———————————————-> Chronic - very little inflammation still happening —. weight loss now occuring along with other symptoms.

SIGNS & SYMPTOMS

0 Cough ( can be non pro (acute)/ productive (chronic / subacute) - depending on phase
0 Dyspnoea
0 fever chills
0 Malaise
0 Bibasilar rales or diffuse rales
0 Clubbing
0 Weight loss (often last sign - in chronic HP)

RISK FACTORS

• Exposure to birds , Cleaning cages - Exposure to avian protein antigen (in excrement )

Farmer , old ventilation / humidifers- Mould in work enviroment

Occupational exposure - working aft factory (machinery , spray pain fro vehicles etc)

• Exposure to mental working fluid.

Question 4

Investiagtion of HP?

Answer 4

PFTs
(bedside test - Spirometry
- can show restrictive pattern (in Acute HP , but mixed restrictive / obstructive pattern in sub acute / chronic . )

• FBC - leukocytosis
• ESR - elevated
• Albumin (may be low in chronic disease )
• CXR
  ( show infiltates , nodular or patchy , fibrosis )
• CT scan - more accurate than CXR ( used in chronic HP - to condirm diagnosis )
• can also help distinguish from other Interstitiual lung diseases )
• Diffusing lung capacity of CO
  (reduced)

Consider

bronchoalveolar lavage ( too look for caustive organism antigens - fluid is analysed)
( show leulocytosis)

Lung biopsy - only done if patient present ewith atypical features of absence of exposure history .

Question 5

Treatment of HP?

Answer 5

1ST LINE

Avoidance of antigen + Corticosteriod - oral pred (& taper dose - reduce over time)

Chronic

1ST LINE

• Avoidance of antigen + long term low dose corticosteriod therapy .( pred oral )

Question 6

How did HP used to be classified ? - just for knowlegde

Answer 6

PHASES - no longer used

• Acute phase ( develops over a few hours after exposure )
• Subacute - develops over weeks to months .
• Chronic - develops over months to years after exposure.

HOW DOES IT PROGRESS.- no longer really used bcc of difficulty differientiating btw stages.

( Acute - Inflammation is at its most intense - Fever, dyspnoea , tachypnoea , pulomary infiltrates (substance denser than air - blood , pus . protein ) , restrictive PFTs , reduced disffusing lung capcity of CO (DLCO) due to lymphocytic alveolitis ———————————————————————————->2. Sub acute ( inflammation reduces in intensity —->Fibrosis is increasing ( insidous development - gradually developing , subtle but very harmful )——> malaise , dyspnae , misxed picture PFT and reduced DLCO ———————————————-> Chronic - very little inflammation still happening —. weight loss now occuring along with other symptoms.

Question 7

What is the difference btw pneumoconiosis & HP ?

ASK DOCTOR WHAT THE DIFFERENCE IS ?

Answer 7

pneumoconiosis - chronic lung disease due to inhalation of mineral dust e.g asbestosis , silicossis , berylliosis, coal workers lung , baritosis , chalicosis ,

HP - inflammation do inhalation of organic dust / partcicles.

Question 8

What is Pneumoconiosis ?

Answer 8

Chronic lung disease caused by inhalation of mineral / metal particles ————————————–> WBC engulf these particles ————-> inflammation ——-> over time
causes fibrosis ———————> Causes lung disease.

TYPES -MOST COMMON

0 Asbestiosis (Asbestos exposure )——————-> increases risk of Lung cancer & Misothelioma

0 Silicosis (Silicon exposure - sand blaster or silician miners )—————-> increases risk of TB.

0 Coal worker’s pneumoconiosis (Black lungs disease ) (Coal exposure)

0 Chronic berylilium disease . (Berylilium exposure)

LESS COMMON

• Aluminosis
• Baritosis
• Graphite pneimoconiosis
• oil shale pneumoconiosis
• Siderosis
• Stannosis
• Talcosis

SIGNS & SYMPTOMS

• Dyspnoea on exretion - common
  (Increases with progression of disease )
• Cough - dry , non productive
  ( frequency increases wirh progression )
• Chest tightness / wheezing ( not common - may occur in patient who have develop COPD alongside.
• IMPORTANT - Chest auscultation is often normal in these disease.
  ( but in chronic berylilium disease - may hear crackles )

As disease progressie & becomes more severe my become/ develop :

• Barrel chest
• Cynaotic
• Weight loss
• Finger clubbing ( only in advance stage )

POSSIBLE COMPLICATION

• RA
  (e. g Caplan’s syndrome/ Rheumatoid pneumoconiosis - Peoplw with RA exposed to silica , abestos)
• Scleroderma

RISK FACTORS

• Exposure to the particles
  (Occupational exposure )
• Smoking - liked to development of obstructive changes in those exposed to silica or coal .

Question 9

Diagnosis of Pneumoconiosis ?

Answer 9

CXR -
may see :
progressive upper zone non - calcified small opacities
(egg shell calcification) - Silicosis , Coal workers

• Presence of multiple (in the hundreds ) in upper zone - highly suggestive of silicosis or coal workers disease.

0 Spirometry- may show normal , restrictive (may show obstructive ? or mixed pattern

Beryllium lymphocyte proliferation test ( BeLPT ) -ESSENTIAL IF you want to diganose chronic berulium exposure

*(those with Silicosis should be tested for TB- Tubercullin skin or blood test , these patients are at risk of developing TB - should be treated if test is positive or 10mm (blood test) or above on the skin test ————> can then do a IGRA , infereron gamma - release assay.
(especially if HIV positive - most vulnerable ) ———————> can do a sputum smear & culture( for TB)

CONSIDER :
- (Bronchoscopic lavage - also rountie for chronic beryllium disease
(not really the best for silica & coal exposure )

• High resolution CT (HCRT)
• Lung biopsy - not really done (rarely needed for diagnosis)

-

Question 10

Treatment of Pneumoconioses ?

Answer 10

ACUTE SILICOSIS
( this is a rare form - similar symptoms to chronic form just occur faster )

1ST LINE - lung lavage (lung washing )
(use of saline to wash out lung

ACUTE BERYLLIOSIS

1ST LINE
- Corticosteriod therapy (prednisolone oral)

CHRONIC

Berylliosis - smoking cessation + removal of occupatioal exposure + oral pred

For all others - most smoking cessation + removal of exposure + :

• oxygen - if hypoxic
• Bronchodilator therpay - if COPD present
• Lung transplant - if end stage lung failure
• Pulmonary rehabilitaion - if external dyspnoea .
• smoking + exposure to mineral dust - BAD

Question 11

What is PAP - Pulmonary alveolar proteinosis ?

Answer 11

Very rare - alveoli stops functioning properly due to build up of surfactant (oil protein substance)

CAUSES - too much is produced and the rate of production is faster than the rate of clearance so lungs cannot get oxygen ———-> Breathlessness , coughing ,pain.

TYPES

0 Primary/ Idiopathic
- PAP(most common)
0 Secondary PAP
- lung cancer
- massive inhalation of some particular types of  dust or fumes 
- Infection (especially the lung)
0 Congenital PAP  - genetic disorder

Question 12

What is Asbestosis ?

Answer 12

Form of Pneumoconioses

Type of intersitiual lung disease ————————————–> asbestosis fibres cause fibrosis of the lung when it is inhaled.

SIGNS / SYMPTOMS

• Dyspnoea
• Cough
• crackles ( usually heard at bases in early disease that progresses through lung fields)
• clubbing
  (can be a sign of fibrosis )

Chest pain - not common (patients can often confuse chest pain with chest tightness from SOB)- severe unremitting chest pain can raise concern about cancer if you are thinking non cardiac cause.

RISK FACTORS

• Occupational exposure ( shipyard worker, maintenance , electricians , construction , production , heating & ventilation workers , plumbers
• Smoking history

COMPLICATIONS

• higher risk of cancers e.g lung cancer , Mesothelioma ( Cancer of the lining of the lungs - in this case (can be the lining of the lungs, tummy , heart or testicles)

Question 13

Diagnosis of asbestosis ?

Answer 13

0 CXR

• pleural changes :
• Pleural thickening (scar tissue develops on the lining of the pleura )

0 PFts 
( restrictive pattern - but may show obstructive especially if asbestos expsosure + smoking)

CONSIDER :

HRCT - high resolution CT - can see pleural thickening better (more sensitive than CXR)
* may see lower interstitual fibrosis

0 Lung biopsy (rarely needed for biopsy ( should only used be used if cancer is suspected or absence of known asbestos exposure )

• Just a note - if patient describes unrelenting chest pain & cardiac cause has been excluded or not likely ——-> think cancer ( if signs of this - as Mesothelioma has chest pain and show pleural thickening on CXR like asbestosis. ) - Pleural thickening can also be caused by lung cancer or metastic cancer . )

Question 14

What should you suspect if a person has risk factors of
Immunosupression( conditions & drugs ) , born in high prevelance areas , children , untreated condition ,
And has weight loss , fever , night sweats , anorexia or malaise?

Answer 14

Active TB should be suspected if a person has risk factors andweight loss, fever, night sweats, anorexia, or malaise.

Pulmonary involvementmay present with persistent productive cough,breathlessness, and haemoptysis.

Extrapulmonary involvementmay present with organ-specific symptoms and signs.

Question 15

Treatment of Asbestosis ?

Answer 15

Cant reverse damage done to lungs

1ST LINE

- Smoking cessation advice + Pulmonary rehabilitation (help with breathlesness , improve exercise tolerance , nutritional support etc. )+ 
oxygen therapy (help reduce risk of pulmonary hypertension thus Cor pulmonale )

PLUS - Supportive care

• if obstructive disease present - give bronchodilators
• if sputum increase , increase SOB , fever (signs of infection) - Give antibitotics

2ND LINE 
Pleural decortication ( removal of a restrictive fibrous tissue layer  overlying the lung )

or

Lung transplant

Question 16

What disease are notifiable disease to public health England?

Have to notify them if suspect or gave diagnosed.

Answer 16

Acute encephalitis

Acute infectious hepatitis

Acute meningitis

Acute poliomyelitis

Anthrax

Botulism

Brucellosis

Cholera

COVID-19

Diphtheria

Enteric fever (typhoid or paratyphoid fever)

Food poisoning

Haemolytic uraemic syndrome (HUS)

Infectious bloody diarrhoea

Invasive group A streptococcal disease

Legionnaires’ disease

Leprosy

Malaria

Measles

Meningococcal septicaemia

Mumps

Plague

Rabies

Rubella

Severe Acute Respiratory Syndrome (SARS)

Scarlet fever

Smallpox

Tetanus

Tuberculosis

Typhus

Viral haemorrhagic fever (VHF)

Whooping cough

Yellow fever

Question 17

How do you work of mean aterial pressure ?

What is it ?

Answer 17

The average pressure in a persons ateries in one cycle .

(Diastolic BP X 2) + systolic BP )/ 3
(divided by 3