Respiratory 2 Flashcards
What are restrictive lung diseases?
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
How does restrictive lung disease affect PFTs ?
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 ?)
What is Hypersensitivity pneumonitis ?
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.
Investiagtion of HP?
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 .
Treatment of HP?
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 )
How did HP used to be classified ? - just for knowlegde
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.
What is the difference btw pneumoconiosis & HP ?
ASK DOCTOR WHAT THE DIFFERENCE IS ?
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.
What is Pneumoconiosis ?
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 .
Diagnosis of Pneumoconiosis ?
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)
-
Treatment of Pneumoconioses ?
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
What is PAP - Pulmonary alveolar proteinosis ?
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
What is Asbestosis ?
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)
Diagnosis of asbestosis ?
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 . )
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?
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.
Treatment of Asbestosis ?
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
