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Clinical > Respiratory: Interstitial and Occupational Lung Disease > Flashcards

Respiratory: Interstitial and Occupational Lung Disease Flashcards

1
Q

What is interstitial disease and symptoms?

A

Any disease process affecting lung interstitium and has a RESTRICTIVE lung pattern, i.e: alveoli, terminal bronchi
Interferes with gas transfer
Symptoms - breathlessness, dry cough

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2
Q

Types of interstitial lung disease, in relation to times?

A 
Acute - sudden
Episodic - comes and goes 
Chronic - comes on gradually and is:
Part of systemic disease
Exposure to agent, e.g: dust, drug, etc
Idiopathic
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3
Q

Categories of ILD?

A

4 main categories:
ILD of known cause, e.g: drugs, or association, e,g:; collagen vascular disease (underlying condition)
Idiopathic Interstitial Pneumonia (IIP)
Granulomatous ILDs, e.g: Sarcoidosis, EAA
Other forms of ILD
ADD PICTURE

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4
Q

What is sarcoidosis?

A

Granulomatous (imbalance of immune system with type IV hypersensitivity) disease of unknown cause, but less common in smokers and common in Afro-Caribbean women
Multiple-system involvement:
Common - lungs, lymph nodes, joints, liver,n, eye
Less common - kidneys, brain, nerves, heart

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5
Q

Granulomas in sarcoidosis?

A

Non-caseating granuloma (not necrotic at centre) of unknown aetiology; probably infective agent in susceptible individuals

Composed of epitheloid histiocytes, multinucleated giant cells, lymphocytes, plasma cells, fibroblasts and collagen

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6
Q

Types of sarcoidosis?

A

Acute

Chronic

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7
Q

Characteristics of acute sarcoidosis?

A

ERYTHEMA NODOSUM - red, painful nodules on legs - new ones appear and old ones fade
Bilateral hilar lymphadenopathy- can be seen on CXR - and lung infiltrates
Arthritis
Uveitis (inflammation of uvea - middle layer of eye), iritis (inflammation of iris), parotitis (inflammation of parotid gland)
Fever

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8
Q

Characteristics of chronic sarcoidosis?

A 
Lung infiltrates (alveolitis)
Skin infiltrations
Peripheral lymphadenopathy
Hypercalcaemia
Other organs - renal, myocardial, neurological, hepatitis, splenomegaly
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9
Q

Diagnosis of sarcoidosis?

A

Pulmonary function - restrictive defect due to lung infiltrates
Blood test for

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10
Q

Differential diagnosis along with sarcoidosis?

A

TB
Lymphoma
Carcinoma
Fungal infection

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11
Q

Sarcoidosis investigations?

A

CXR (bilateral hilar lymphadenopathy)
CT scan of lungs
Tissue biopsy, e.g: transbronchial, skin, lymph node (non-caseating granuloma)

Blood test - Angiotensin Converting Enzyme (ACE) levels as activity marker (NOT DIAGNOSTIC), raised calcium, increased inflammatory markers

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12
Q

Treatment of sarcoidosis?

A

Acute - self-limiting condition, usually no treatment unless vital organs affected, e.g: lungs, eyes, heart, brain (give STEROIDS)
Chronic - oral steroids usually needed and immunosuppression, e.g: azathioprine, methotrexate, anti-TNF therapy

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13
Q

Cautions with sarcoidosis after treatment?

A

Monitor with CXR and pulmonary functions tests for several years - often relapses

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14
Q

What is extrinsic allergic alveolitis?

A

AKA lymphocytic alveolitis/hypersensitivity pneumonitis (pathological term)

TYPE III HYPERSENSITIVITY (immune complex deposition - patient exposed to antigens and forms immune complexes)
Alveolitis - inflammation of the alveoli

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15
Q

Causes of extrinsic allergic alveolitis?

A

Thermophilic actinomycetes (farmers’ lung - reaction to fungus in mouldy hay - , malt workers, mushroom workers), avian antigens (bird fanciers lung), drugs (gold, bleomycin, sulphasalazine)

No causes identified in ~30% of cases

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16
Q

Types of extrinsic allergic alveolitis?

A

Acute

Chronic - repeated low dose antigen exposure over time (years)

17
Q

Symptoms of acute extrinsic allergic alveolitis?

A 
Classically occur SEVERAL HOURS AFTER acute exposure (flu-like illness) 
Cough
Breathless
Fever
Myalgia
Hypoxia
Crackles (NO WHEEZE)

CXR - widespread pulmonary infiltrates

18
Q

Treatment of acute extrinsic allergic alveolitis?

A

Treatment:
Oxygen
Steroids
Antigen avoidance

19
Q

Symptoms of chronic extrinsic allergic alveolitis?

A

Progresses breathlessness and cough
Crackles
Clubbing is unusual

20
Q

Investigations for chronic extrinsic allergic alveolitis?

A

Pulmonary functions tests show restrictive defects (low FEV1 and FVC, high/normal ratio, low gas transfer - TLCO)

CXR PULMONARY FIBROSIS, commonly in upper zones

21
Q

Diagnosis of chronic extrinsic allergic alveolitis?

A

History of exposure
Precipitins (IgG antibodies to guilty antibodies)
Lung biopsy if in doubt

22
Q

Treatment of chronic extrinsic allergic alveolitis?

A

Remove antigen exposure
Oral steroids if breathless/low gas transfer
May have to give immunosuppressants

23
Q

What is Idiopathic Pulmonary Fibrosis (IPF)?

A 
Most common ILD - lung trying to repair itself, lays down scar tissue, when it does not need to be repaired
Unknown aetiology; could be:
Imbalance of fibrotic repair system
Related to gastric reflux
IPF is NOT an inflammatory disease
More common in smokers
24
Q

Pathology of IPF?

A

Chronic inflammatory infiltrate (neutrophils and fibrosis in alveolar walls +/- intra-alveolar macrophages)

25
Q

Secondary causes of pulmonary fibrosis?

A

Rheumatoid
Systemic Lupus Erythematosus (SLE)
Systemic sclerosis
Asbestos exposure

Drugs - nitrofurantoin, bleomycin, penicillamine

26
Q

Clinical presentation of IPF?

A

Progressive breathlessness over several years

Dry cough

27
Q

What is found on examination of a patient with IPF?

A

Clubbing

Bilateral fine inspiratory crackles

28
Q

Investigations for IPF?

A

Restrictive defect on PFTs - reduced FEV1 AND FVC (with normal/raised FEV1/FVC ratio), reduced lung volumes, low gas transfer

CXR - bilateral infiltrates

CT scan - reticulonodular fibrotic changes with HONEYCOMBING (worse at lung bases); presence of “ground-glass” suggests reversible alveolitis (FIBROSIS IS IRREVERSIBLE)

Lung biopsy (wither transbronchial/thoracoscopic) - not necessary if CT scan is diagnostic

29
Q

Differential diagnosis along with IPF?

A 
Exclude occupational diseases, like asbestosis and silicosis
Mitral valve disease
Left ventricular failure
Sarcoidosis
Extrinsic allergic alveolitis

Often get diagnosed with heart failure mistakenly

30
Q

Diagnosis of IPF?

A

Ask about occupation (in depth), pets and drugs

Diagnosis - combo of history, examination and radiology tests

31
Q

Treatment of IPF?

A

Mostly ineffective and aimed at slowing down progression than than reversing fibrosis - steroids and immunosuppressants do not change course of disease
N-acetyl cisteine may have anti-fibrotic effects via anti-oxidant properties
Pirfenidone - (not anti-inflammatory) new anti-fibrotic drug (only therapy shows in randomised controlled trials to slow down disease progression but very expensive and has a lot of side effects

O2 if hypoxic
Lung transplantation in young patients

Future treatments: other anti-fibrotic agents, pulmonary artery vasodilators (as some develop pulmonary hypertension)

32
Q

IPF prognosis?

A

Most patients progress into respiratory failure and are dead within 5 years

33
Q

Types of coal workers pneumoconiosis?

A

Simple - CXR abnormality only (no impairment of lung function - often associated with COPD)

Complicated - progressive massive fibrosis leads to a restrictive pattern with breathlessness

Not malignant but ball of fibrous tissue form and take over lung, leading to death

Often associated with chronic bronchitis (unsure if this is coal dust or smoking)

Caplan’s syndrome - rheumatoid pneumoconiosis, pulmonary nodules (rheumatoid arthritis + coal workers pneumoconiosis)

Baritosis (exposure to barium) can look similar

34
Q

Describe silicosis

A

Usually due to 15-20 years exposure to quartz, e.g: mines
A form of simple pneumoconiosis (CXR abnormality only with egg-shell calcification of hilar nodes)

Chronic silicosis - leads to restrictive pattern and pulmonary fibrosis

35
Q

Asbestos related lung conditions?

A 
Occupations in mining, ship building, boilers, piping, automotive components
Include:
Pleural disease
Pulmonary fibrosis
Bronchial carcinoma
36
Q

Types of asbestos related pleural disease?

A

Benign pleural plaques - asymptomatic
Acute asbestos pleuritis - fever, pain, bloody pleural effusion
Pleural effusion and diffuse pleural thickening (restrictive impairment)
Malignant mesothelioma - incurable pleural cancer that present with chest pain and pleural effusion and is ONLY CAUSED BY ASBESTOS, even if exposure was a long time ago; no available treatment - fatal within 2 years

37
Q

Describe asbestos related pulmonary fibrosis

A 
AKA ASBESTOSIS (heavy prolonged exposure); asbestosis is not pleural disease, it is pulmonary fibrosis 
Diffuse pulmonary fibrosis and restrictive defect 
Asbestos bodies in sputum and fibres in lung biopsy
38
Q

CXR of asbesotis

A

Looks like pulmonary fibrosis; diffuse interstitial changes to both lungs
Plaques can be seen on both CXR and CT scan

39
Q

CXR of malignant mesothelioma?

A

Pleural thickening impairs gas exchange

Clinical (54 decks)

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