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Respiratory > Restrictive Lung Disease > Flashcards

Restrictive Lung Disease Flashcards

1
Q

What is pulmonary fibrosis?

A

Excess collagen/scar tissue in the interstitial tissue of the lung

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2
Q

What are the causes of pulmonary fibrosis?

A

Occupation

  • Exposure to pets/birds EAA (extrinsic allergic alveolitis)

Previous radiotherapy

Drugs

  • Amiodarone
  • Methotrexate
  • Nitrofurantoin
  • Penicillamine
  • Bleomycin
  • Cyclophosphamide

Systemic disorders

  • Vasculitis
  • Wegener’s granulomatosis
  • Churg–Strauss syndrome
  • Behçet’s syndrome
  • Goodpasture’s syndrome
  • Sarcoidosis

Connective tissue disorders

  • SLE
  • Rheumatoid arthritis
  • Sjögren’s syndrome
  • Systemic sclerosis
  • Polymyositis

Neoplasm

  • Lymphoma
  • Lymphangitic carcinoma

Inherited disorders

  • Tuberous sclerosis
  • Neurofibromatosis

Others

  • Amyloidosis
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3
Q

What drugs can cause pulmonary fibrosis?

A

Amiodarone

Methotrexate

Nitrofurantoin

Penicillamine

Bleomycin

Cyclophosphamide

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4
Q

What investigations are used in pulmonary fibrosis diagnosis?

A

Bloods

  • FBC
  • U&E
  • LFT
  • Calcium levels
  • ACE (angiotensin-converting enzyme) levels

Immunology

  • Anti-GBM (glomerular basement membrane)
  • ANA (antinuclear antibodies)
  • ANCA (antineutrophil cytoplasmic antibodies)
  • Rheumatoid factor
  • Serum precipitins

Imaging

  • CXR
  • HRCT

Spirometry

  • Restrictive pattern

Bronchoscopy + biopsy

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5
Q

Describe a restrictive pattern in spirometry

A

decreased FEV1

decreased FVC

normal or increased FEV1/FVC

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6
Q

Describe the presentation of idiopathic pulmonary fibrosis

A

Clinically slow progression

Cough

Dyspnoea

Clubbing

Fine bi basal end inspiratory crackles

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7
Q

What is the prognosis of idiopathic pulmonary fibrosis?

A

poor, average life expectnancy 3-4 years

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8
Q

What is the management of idiopathic pulmonary fibrosis?

A

Pulmonary rehabilitation

Oral Anti-Fibrotic Drugs (OAF)

  • Pirfenidone

Lung transplant

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9
Q

What is Sarcoidosis?

A

Chronic multisystem non-caseating granulomatous inflammatory disorder, most commonly affecting the lung

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10
Q

Describe the presentation of sarcoidosis

A

Depends on the organs involved

Dyspnoea

Dry cough

Erythema Nodosum

  • Raised marks on legs

Red eyes

Redness around scars

Lupus pernio pertains

  • Rash, not normally itchy or painful, affecting nose, lips, cheek, ears and digits

Arthralgia

Bilateral Hilar Lymphadenopathy

Systemic

  • Fever
  • Malaise
  • Weight loss
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11
Q

What investigations are used in the diagnosis and monitoring of sarcoidosis?

A

CXR

>Ca2+ Hypercalcaemia

  • Macrophages inside the granulomas cause an increased conversion of vitamin D to its active form

Bronchoscopy and biopsy

  • Red
  • Granulomas

Eye Examination

  • Assess for fibroids in back of eye

>Serum ACE levels

  • Poorly specific but can be used to monitor disease activity
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12
Q

What causes sarcoidosis?

A

Idiopathic

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13
Q

What is the management of sarcoidosis?

A

No treatment if mild disease

NSAIDS

Steroids

For resistant disease consider

  • Methotrexate
  • Chloroquine
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14
Q

Name some possible complications of sarcoidosis?

A

Pneumothorax

Bronchiectasis

Respiratory failure

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15
Q

What CXR signs are seen in Sarcoidosis?

A

Large hilum area/bilateral hilar lymphadenopathy

Snow storm lungs

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16
Q

What group is sarcoidosis more common in?

A

Afro-Carribean ethnicity

F>M

17
Q

What are the indications for steroid use in sarcoidosis?

A

Parenchymal lung disease

Uveitis

Hypercalcaemia

Neurological or cardiac involvement

18
Q

What is the prognosis of sarcoidosis?

A

Sarcoidosis spontaneously resolves within 6 months in around 60% of patients

In a small number of patients it progresses with pulmonary fibrosis and pulmonary hypertension, potentially requiring a lung transplant

Death in sarcoidosis is usually when it affects the heart (causing arrhythmias) or the central nervous system

19
Q

What is hypersensitivity pneumonitis?

A

Immunological disease characterised by hypersensitivity to various antigens resulting in a allergic reaction, also known as extrinsic allergic alveolitis (EAA)

20
Q

Describe the presentation of hypersensitivity pneumonitis?

A

Symptoms usually regress when causative agent is removed

Dry cough

Pyrexia

Myalgia

Rigors 4-9 hours after antigen exposure

Tachypnoea

Auscultation

  • Wheeze
  • Crackles
21
Q

What type of hypersensitivity reaction is involved in hypersensitivity pneumonitis?

A

Type 3 and 4 hypersensitivity reaction

22
Q

What are the causes of hypersensitivity pneumonitis?

A

Chemicals

Bird/animal proteins (Avian protein)

Fungi

Bacteria

Thermactinomyces in mouldy hay

23
Q

What causes ‘bird lung’?

A

Avian protein on bird feathers

24
Q

What causes farmers lung?

A

Thermactinomyces in mouldy hay

25
Q

What is the management of hypersensitivity pneumonitis?

A

Corticosteroids

Avoid antigen

Respiratory (14 decks)

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