Uoa Resp - Diffuse Parenchymal Lung Dis (Ild)

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Diffuse Parenchymal Lung Disease COMMONLY known as

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ILD

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ILD define

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Inflammation of the pulmonary interstitium with or without progression to fibrosis

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ILD includes how many different diseases

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Over 200 different diseases some of which are systemic and others confined to the lungs

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causes of ILD

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• Idiopathic e.g. usual interstitial pneumonia
• Environmental e.g. asbestosis
• Drug or toxin e.g. amiodarone
• Secondary to connective tissue disease e.g. nonspecific interstitial pneumonia
• Malignant e.g. lymphangitis carcinomatosis
• Genetic e.g. sarcoidosis, in part

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ILD presenting symptoms and signs

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• Insidious onset of exertional dyspnoea and dry cough

• Wheeze is not a feature!

• Clubbing may be present

• Cyanosis may be present in advanced stages

• Auscultation reveals basal ‘velcro’crackles in many patients

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do the sxs and signs of ILD help to differentiate b/w different ILD

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NO

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ILD physiology - think small stiff lungs

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• Reduced lung volumes

• Preserved FEV1/VC ratio

• Reduced diffusion (DLCO)

• Reduced total lung compliance

• Widened A-a oxygen gradient

• Respiratory limitation on exercise

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ILD Making the Diagnosis:

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History and examination

• HRCT – looking for key morphologic features and extent of disease

• Bronchoscopy: bronchoalveolar lavage, transbronchial biopsy

• Thoracoscopic lung biopsy

• Biopsy of other tissue e.g. peripheral lymph node in sarcoidosis

HRCT- high resolution CT

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are blood tests generally diagnostic in ILD

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NO - rarely they are

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Principles of management of ILD

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-try get dx

• Ensure baseline staging of disease severity with HRCT and lung function tests
• Establish if disease responsive to treatment
• If there is an inflammatory basis and treatment is indicated then steroids or immunosuppressants may be prescribed e.g. in sarcoidosis
• If causative exposure applies then remove e.g. in EAA.
• Where no effective treatment exists consider enrolling patients in clinical trials e.g. IPF
• Undertake regular restaging to monitor response to treatment.
• For many ILDs there is no specific pharmacologic treatment and it may be appropriate to consider transplant or palliative care.

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Pleural effusion definition

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is excess fluid that accumulates in the pleural cavity, the fluid-filled space that surrounds the lungs. This excess can impair breathing by limiting the expansion of the lungs

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PLEURAL EFFUSION can be due to

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Increased entry rate (30 x normal) or

reduced exit rate

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PLEURAL EFFUSION can be due to

Increased entry rate (30 x normal) resulting in

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transudate or

exudate

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PLEURAL EFFUSION can be due to

reduced exit rate eg

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lymphatic obstruction

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PLEURAL EFFUSION can be due to

Increased entry rate (30 x normal) resulting in

transudate can be due to:

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either:

↑ hydrostatic pressure e.g. heart failure or

↓ oncotic pressure e.g. cirrhosis, nephrotic syndrome

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PLEURAL EFFUSION can be due to

Increased entry rate (30 x normal) resulting in

transudate, is there any problem/abnormality with the pleural membrane

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no, theres no abn. of pleural membrane

nb transudate is proportional to preload - what does that mean?

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PLEURAL EFFUSION can be due to

Increased entry rate (30 x normal) resulting in

exudate due to:

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increased permeability of the pleural membrane ie increased K+

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PLEURAL EFFUSION can be due to

Increased entry rate (30 x normal) resulting in

exudate due to increased permeability of the pleural membrane - what causes this increased permeability?

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due to infection and inflammation

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PLEURAL EFFUSION can be due to

Increased entry rate (30 x normal) resulting in

exudate due to increased permeability of the pleural membrane due to infection and inflammation - what causes the inflammation:

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e.g.

- infection

- malignancy

- inflammation

- vascular

- GI problems

- drugs

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what is used to determine if a pleural effusion is exudative vs transudative

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Light’s Criteria

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according to the lights criteria a pleural effusion is likely exudative if at least one of the following exists:

nb

sensitivity and specificity of Light's criteria for detection of exudates around 98% and 80%, respectively

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- pleural protein : plasma protein > 0.5

- pleural LDH : plasma LDH > 0.6

- pleural LDH > 2/3 of the normal upper limit for serum

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signs of pleural effusion

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- dyspnoea

- cough

- pleuritic chest pain

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in pleural effusion the decrease in lung volume is how much

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about 1/3 of the normal volume

but little change in gas exchange

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with pleural effusion despite the decrease in lung volume by 1/3 is there any significant changes in gas exchange

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NO

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Pleural effusion

Investigations:

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CXR

pleural aspiration

closed pleural biopsy

imaging

bronchoscopy

thoracoscopy/open biopsy

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Pleural effusion

Investigations: CXR details

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detect 200mls on PA,

50mls on lateral

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Pleural effusion

Investigations: do you always need a pleural aspirate

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not necessary in bilateral effusions and if transudate strongly suggested

this suggests aspirates are mainly for exudate suspicion (toe's note)

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Pleural effusion

Investigations: pleural aspirations are useful for

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diagnostic purposes and to relieve symptoms

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Pleural effusion

Investigations: pleural aspirations are used for diagnostic purposes, what especially are you looking for

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- appearance

- protein

- glucose

- pH ( in blood gas syringe, if <7.2 need to insert tube)

- WCC and differential ( PMN vs lymphocytic vs eosinophilic)

- Gm stain and culture ( include for AFB)

- Cytology ( 60-70% positive in malignancy)

- Amylase (pancreatitis vs oesophageal rupture)

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pleural aspiration is aka

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thoracentesis, thoracocentesis, pleural tap

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Thoracentesis is used diagnostically to establish the

thoracentesis video

http://www.nejm.org/doi/full/10.1056/NEJMvcm053812

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cause of a pleural effusion.

It can also be performed to drain large effusions that lead to respiratory compromise

do not put it below the 9th rib

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whats the AFB test/screen/smear

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Acid-Fast Bacillus Smear and Culture and Sensitivity - looking for active TB

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should you do a AFB test on a pleural aspiration

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yes, to look for active TB

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for a pleural aspirate at what pH would consider inserting a tube

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if pH was less than 7.2 (need to insert tube)

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review details around:

WCC and differential ( PMN vs lymphocytic vs eosinophilic)

Cytology ( 60-70% positive in malignancy)

Amylase (pancreatitis vs oesophageal rupture)

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review details around:

WCC and differential ( PMN vs lymphocytic vs eosinophilic)

Cytology ( 60-70% positive in malignancy)

Amylase (pancreatitis vs oesophageal rupture)

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InV for pleural effusion

why do a closed pleural biopsy

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if suspect TB and malignancy

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InV for pleural effusion

what imaging would you do

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U/S – fluid, septations

CT – with contrast +/- biopsy

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InV for pleural effusion

CT – with contrast +/- biopsy

when would you consider doing a biopsy after revieiwing the CT

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if you saw/suspected nodular pleural thickening, mediastinal pleural thickening and circumferential pleural thickening

in other words nodular pleural thickening

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InV for pleural effusion

are bronchoscopy very effective

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Bronchoscopy ( low yield)

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PNEUMOTHORAX define

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air in pleural space

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spontaneous PNEUMOTHORAX classification

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Primary or

Secondary

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details of primary pneumothorax - is associated with underlying lung disease

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No - cause unknown

possible role of sub pleural blebs

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The cause of primary spontaneous pneumothorax is unknown, but established risk factors include

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tall thin males, smoking, and a family history of pneumothorax

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whats a sub pleural blebs

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are small subpleural thin walled air containing spaces, not larger than 1-2 cm in diameter. Their walls are less than 1 mm thick. If they rupture, they allow air to escape into pleural space resulting in a spontaneous pneumothorax

its like a small bullae

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is secondary spontaneous pneumothorax associated with underlying lung disease

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yes, most commonly COPD 70%

e.g. obstructive (COPD, asthma), restrictive (ILD), cavitatory (TB, cancer)

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full list of causes for secondary spontaneous pneumothorax associated with underlying lung disease

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Diseases of the airways[1]

COPD (especially when emphysema and lung bullae are present), acute severe asthma,cystic fibrosis

Infections of the lung[1]

Pneumocystis pneumonia (PCP), tuberculosis, necrotizing pneumonia

Interstitial lung disease[1]

Sarcoidosis, idiopathic pulmonary fibrosis, histiocytosis X, lymphangioleiomyomatosis(LAM)

Connective tissue diseases[1]

Rheumatoid arthritis, ankylosing spondylitis, polymyositis and dermatomyositis, systemic sclerosis, Marfan's syndrome and Ehlers–Danlos syndrome

Cancer[1]

Lung cancer, sarcomas involving the lung

Miscellaneous[2]

Catamenial pneumothorax (associated with the menstrual cycle and related toendometriosis in the chest)

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spontaneous pneumothorax can also be caused by

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traumatic (include iatrogenic) pneumothoraces

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pneumothorax pathophys

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loss of negative intra-pleural pressure

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Primary pneumothorax features

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- young (25-35 years),

- M > > F, body habitus (tall and thin)

- smokers (in >90%),

- familial tendency

- timing of occurrence (usually at rest or normal activities)

- ? role of sub-pleural blebs (80-90%)

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pneumothorax sxs

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- chest pain (?mechanism)

- shortness of breath

- med/s-c emphysema,

- sensation in chest

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Signs of pneumothorax

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reduced expansion and BS

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signs of tension PTX

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distress, tachycardia, tachypnoea, shift of med to opp side, absent BS on affected side

sxs and signs may be subtle – especially with pre-existing lung disease

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signs of tension PTX due to

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hypoxia and reduced cardiac output

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pneumothorax imaging

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CXR

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on CXR for pneumothorax

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PTX vs bulla (shape, role of CT)

nb In some lung diseases, especially emphysema, it is possible for abnormal lung areas such as bullae (large air-filled sacs) to have the same appearance as a pneumothorax on chest X-ray, and it may not be safe to apply any treatment before the distinction is made and before the exact location and size of the pneumothorax is determined - which a CT can help with

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some bullae due to underlying emphysema might look like pneumothorax - what is the complication for CXR and CT

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CXR for this reason may miss the bullae, or pneumothorax altogether

but CT might pick it up (incl. any bullae)

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CXR classification of size of pneumothorax and other relevant features

The size of the pneumothorax (i.e. the volume of air in the pleural space) can be determined with a reasonable degree of accuracy by measuring the distance between the:

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chest wall and the lung

small <2cm vs vs large >=2cm rim between lung and chest wall.

relevance of adhesions/pleural fluid

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what does a pneumothorax size of 2cm represent

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An air rim of 2 cm means that the pneumothorax occupies about 50% of the hemithorax

ie 2cm rim = 50% volume of thorax

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whats the mediastinum

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the structure between the lungs that contains the heart, great blood vessels and large airways

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usefulness of CT scans in pneumothorax

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CT scanning can provide a more accurate determination of the size of the pneumothorax, but its routine use in this setting is not recommended

Accurate pneumothorax size calculations are best achieved by CT scanning

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whats the ideal CXR view for pneumothorax

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PA but if you can't see it, get a lateral view

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pneumothorax mgmt depends upon

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- evidence of tension or respiratory failure

- symptoms (dyspnoea)

- underlying lung disease

- size of PTX

- social circumstances

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Tx options for pneumothorax

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Nil/Observation, ± supplemental O2 (4x absorption rate)

Aspiration

Insertion of ICT ( small (10-14Fr) vs Large (20-24FR) ?with UWSD

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Tx options for pneumothorax

when would you use

Nil/Observation, ± supplemental O2 (4x absorption rate)

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- consider if no underlying lung disease, small (,2cm rim) or not SOB, social circumstances allow

- resolves at 1.25% /day

- underused option

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Tx options for pneumothorax

when would you use

Aspiration

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- more rapid resolution

- not for 2o PTX

- size is not contra-indication

- technique; not without complications

- definition of failure (>3l aspirated) – relates to age, 2o PTX.

- ?observation time

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Tx options for pneumothorax

when would you use

Aspiration - should it be used in 2o PTX

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no

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is size of PTX a CI in aspiration

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no

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ICT =

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INTERCOSTAL CATHETER

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Tx options for pneumothorax

when would you use

Insertion of ICT ( small (10-14Fr) vs Large (20-24FR) ? with UWSD (under water sealed drains)

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- if underlying lung disease (2o PTX)

- technical competence is essential

- attach to UWSD (and suction ?after 24 hours),

- use of Heimlich valve

- allows rapid re-expansion

- do NOT clamp tubes when bubbling, or during transfer.

- Do not advance tubes after insertion

- ? clamp tube after bubbling stops and before removal.

- No indication for chemical pleurodesis

- surgical pleurodesis (open as thoracoscopy). Acute - ongoing air leak eg after 5/7 bubbling, or Elective( second ipsi-, first contra-, bilateral, professional reasons) .

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Tx options for pneumothorax

Insertion of ICT ( small (10-14Fr) vs Large (20-24FR) ? with UWSD (under water sealed drains)

is it used if no underlying disease

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no, its if if underlying lung disease (2o PTX)

technical competence is essential to do it

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Tx options for pneumothorax

Insertion of ICT ( small (10-14Fr) vs Large (20-24FR) ? with UWSD (under water sealed drains)

extra attachments

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- attach to UWSD (and suction ?after 24 hours),

- use of Heimlich valve

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Tx options for pneumothorax

Insertion of ICT ( small (10-14Fr) vs Large (20-24FR) ? with UWSD (under water sealed drains)

whats the purpose or pros of this

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allows rapid re-expansion

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Tx options for pneumothorax

Insertion of ICT ( small (10-14Fr) vs Large (20-24FR) ? with UWSD (under water sealed drains)

what not to do

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- do NOT clamp tubes when bubbling, or during transfer.

- Do not advance tubes after insertion

- ? clamp tube after bubbling stops and before removal

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Tx options for pneumothorax

Insertion of ICT ( small (10-14Fr) vs Large (20-24FR) ? with UWSD (under water sealed drains)

what does No indication for chemical pleurodesis mean

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chemical pleurodesis =

obliterating the pleura (parietal and visceral) with chemical agents e.g. bleomycin, tetracycline

No indicxn for this

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Tx options for pneumothorax

Insertion of ICT ( small (10-14Fr) vs Large (20-24FR) ? with UWSD (under water sealed drains)

indication for surgical pleurodesis

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(open as thoracoscopy).

Acute - ongoing air leak eg after 5/7 bubbling, or Elective (second ipsi-, first contra-, bilateral, professional reasons)

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whats pleurodesis

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Pleurodesis is a medical procedure in which the pleural space is artificially obliterated. It involves the adhesion of the two pleurae

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Complications (of PTX and/or management!)

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- failure of lung to re-inflate

- ongoing air leak

- reduced lung compliance

- endobronchial obstruction

- blocked/misplaced tube

- subcutaneous emphysema

- hemothorax

- pain

- (re-expansion pulmonary oedema)

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Indications for referral to Respiratory Physician

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- on-going air leak (> = 2 days)

- failure of lung to re-expand (> = 1 day)

- worsening subcutaneous emphysema

- re-expansion pulmonary oedema

- development of pleural effusion

- recurrent or bilateral PTX

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Complications (of PTX and/or management!)

- failure of lung to re-inflate

- ongoing air leak

- reduced lung compliance

true/false

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true

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Complications (of PTX and/or management!)

- endobronchial obstruction

- blocked/misplaced tube

- subcutaneous emphysema

true/false

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true

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Complications (of PTX and/or management!)

- hemothorax

- pain

- (re-expansion pulmonary oedema)

true/false

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true

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Recurrence rates:

on same side

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20% after 1, 40% after 2, 80% after 3;

(or = 50% at 4 years)

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Recurrence rates:

on opposite side

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- recurrence is greatest in subsequent few months (6 – 24/12)

- more likely if continue to smoke and have subpleural blebs

- avoid scuba diving and ?sky diving

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SYMPTOMS & SIGNS OF CHRONIC RESPIRATORY DISEASE

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Persistent Symptoms

Poor growth, especially deficient weight gain

Finger clubbing

Chest deformity