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Interstitial lung diseases > Matthew McCabe > Flashcards

Matthew McCabe Flashcards

1
Q

What is the other name for alveolar cells?

A

Pneumocytes

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2
Q

Describe type I pneumocytes

A

‘Wallpaper’ of the alveoli. Simple Squamous Epithelial cells- make up ~90% of the surface area with minimal thickness. Maximises gas diffusion

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3
Q

Describe type II pneumocytes

A

‘Clara cells’. Polygonal in shape, have microvilli on surface and excrete pulmonary surfactant

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4
Q

What are ‘Dust cells’?

A

Free floating alveolar macrophages. Phagocytose any particles which have not been trapped by mucous

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5
Q

What are the two gross stages of ILS?

A

1) Alveolitis- acute inflammatory stage

2) Fibrosis- chronic fibrotic stage

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6
Q

What are the different types of ILS we need to know?

A

1) Fibrosing Alveolitis= Idiopathic Pulmonary Fibrosis= Interstitial Pneumonia
2) Sarcoidosis
3) Extrinsic Allergic Alveolitis
4) Pneumoconiosis

Then ILS assocaited with Connective Tissue diseases

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7
Q

What are the key PATHOLOGY points of Fibrosing alveolitis/ Idiopathic Pulmonary Fibrosis

A

Idiopathic
Characterised by sub pleural & basal fibrosis
Causes ‘dilated air spaces with surrounding fibrosis’
LATE STAGE histology shows ‘Honeycombing’ of lung. Microscopically we see ‘punched out lesions’

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8
Q

What are the key CLINICAL points of Fibrosing alveolitis/ Idiopathic Pulmonary Fibrosis

A 
FINE END INSPIRATORY CRACKLES 
Arthralgia 
weight loss/ fatigue 
exertional dyspnoea  
Ground glass appearance ON X RAY IN EARLY STAGES
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9
Q

What tests do we use to investigate IPF?

A

1) Spirometry, Chest X-ray, Full blood works, CT chest,
If these dont work then try:
2) Bronchial alveolar lavage or trans bronchial biopsy

DTPA can be used to show disease activity once diagnosis is made

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10
Q

What is the management plan of IPF?

A

No cure. treat symptoms with:
Oxygen, Pulmonary Rehab & Opiates
Consider Lung Transplant

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11
Q

What inflammatory cells are present in the different stages of ILS?

A

if we carry out a bronchial-alveolar lavage:
Alveolitis stage= lymphocytes= good prognosis
Fibrosis stage= neutrophils/ eosinophils = poor prognosis

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12
Q

What are the Buzzwords for a Sarcoidosis Presentation?

A 
20-40 yo Afro Caribbean women 
Hilar Lymphadenopathy 
Erythema Nodusum 
Dry cough/ breathless/ chest pain 
Splenomegaly
Keratoconjuctivitis Sicca 
Hypercalcaemia / Hyperuricaemia +/- renal stones
Phalangeal cysts
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13
Q

What is the pathophysiology of Sarcoidosis?

A

A granulomatas disease which causes the formation of non-caseating granulomas throughout many parts of the body.

α 1 hydroxylase enzyme is found in sarcoidosis granules. This means that the calcidiol is converted to calcitriol in sarcoidosis granules- causing osteoclast activation- hence the phalyngeal cysts & hypercalcaemia

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14
Q

What tests do we use for Sarcoidosis?

A 
Blood for Hypercalcaemia 
Slit lamp for occular involvement 
Chest X ray & spirometry (will show restrictive pattern)
X ray of hands for phalyngeal cysts 
Ultrasound to show splenomegaly
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15
Q

What is Sarcoidosis treatment?

A

1) Prednisolone (40-60mg for first 6 weeks, then taper down over 1 year)
2) Immunosuppressants (methotrexate, ciclosporin, cyclophosphamide)
3) Infliximab
4) Lung Transplant in unretractable cases

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16
Q

What is the other name for Extrinsic Allergic Alveolitis

A

Hypersensitivity Pneumonitis

17
Q

What is the generalised mechanism of the umbrella terms Extrinsic Allergic Alveolitis/ Hypersensitivity Pneumonitis?

A

EAA/ HP are conditions in which sensitised individuals undergo pulmonary hypersensitivity reactions to seemingly ‘normal’ inhaled allergens.

18
Q

What are the two stages of Extrinsic Allergic Alveolitis/ Hypersensitivity Pneumonitis?

A

In the acute phase, the alveoli are infiltrated with acute inflammatory cells.

With chronic exposure to the allergen(s) there is granuloma formation and IRREVERSIBLE obliterative bronchiolitis (the complete blockage of small airways)

19
Q

What types of hypersensitivity reactions are responsible for Extrinsic Allergic Alveolitis/ Hypersensitivity Pneumonitis?

A

Types II & III

20
Q

What are the 4 types of Extrinsic Allergic Alveolitis/ Hypersensitivity Pneumonitis and their respective allergens?

A
  • Thermophilic bacteria – Farmers lung.
  • Avian proteins – Bird fanciers lung
  • Aspergillus Clavatus =Malt workers lung
  • Thermoactinomyces Sacchari = Sugar worker’s lung
21
Q

What are the Acute vs Chronic Symptoms & tests for EAA/ HP?

A

Acute= Fevers, rigors, dyspnoea, DRY COUGH. Chest X ray shows UPPER ZONE consolidation & restrictive spirometry during periods of exposure

Chronic= weight loss, Exertional dyspnoea. Chest X ray shows UPPER ZONE fibrosis & permanent restrictive spirometry

22
Q

Treatment of EAA/ HP

A

Avoid allergen. In chronic cases prednisolone & O2 may be required

23
Q

What does the umbrella term Pneumoconiosis represent?

A

Pneumoconiosis is the name given to a set of occupational lung diseases which is caused by dust being inhaled into the lungs over a prolonged period of time (>15 years).

24
Q

What is the pathophysiology of Pneumoconiosis?

A

Dust particles are inhaled and digested by dust cells. Upon breakdown, macrophages release inflammatory enzymes and IL-1 which active fibroblasts which increase collagen deposition- thus causing fibrosis.

25
Q

What are the different types of Pneumoconiosis and what dust particles are responsible for each type?

A

1) Coal worker’s Lung- caused by carbon dust
2) Asbestosis — asbestos fibres
3) Silicosis (potter’s rot) -crystalline silica dust

26
Q

What are the buzzwords for Coal workers lung?

A

Asymptomatic chronic bronchitis
CXR will show MULTIPLE SMALL ROUND OPACITIES (1-10mm) IN UPPER ZONES OF THE LUNGS ( be aware rheumatoid conditions can present with nodules so history is key here)

27
Q

What is the most fatal complication of ANY untreated Pneumoconiosis?

A

Progressive Massive Fibrosis (most commonly seen in cases of coal workers lung). Fibrosis of entire lung- fibrotic masses arising in upper zone & up to 10cm diameter

28
Q

What are the buzzword X ray findings for silicosis?

A

CXR SHOWS EGG SHELL CALCIFICATION OF HILAR LYMPH NODES AND DIFFUSE NODULES IN UPPER ZONES IN LUNGS

29
Q

How many types of Asbestos fibres are there and how dangerous is each type?

A

1) White fibres- least fibrogenic
2) Brown fibres- very rare and have intermediate fibrogenicity
3) Blue fibres- most fibrogenic

30
Q

What are the 2 key clinical features of asbestosis?

A

Pleural plaques

Fine end respiratory crackles

31
Q

What is mesothelioma?

A

Cancer of the mesothelial cells lining either the visceral pleura or the visceral pertioneum of other organs (rare) 99% of cases will have asbestos in the history. Mesothelioma can occur up to 45yrs after exposure.

32
Q

How do we diagnose mesothelioma?

A

1) CXR/ CT shows pleural thickening &/or effusions
2) If Pleural fluid is aspirated- Blood will be present
3) Pleural biopsy is gold standard

33
Q

Management for Mesothelioma?

A

Can use chemo but realistically. Compensation & a coffin.

34
Q

What is the other name given to Interstitial Lung Disease?

A

Diffuse Parenchymal Lung Disease

Interstitial lung diseases (1 decks)

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