Interstitial lung disease Flashcards Preview

Cardio-Resp > Interstitial lung disease > Flashcards

Flashcards in Interstitial lung disease Deck (21)
Loading flashcards...
1

What conditions are encompassed within interstitial lung disease?

  • Idiopathic interstitial pneumonia
    • Usual Interstitial Pneumonia (UIP)
    • Non-specific Interstitial Pneumonia (NSIP)
  • Extrinsic Allergic Alveolitis
  • Sarcoidosis
  • Other conditions

2

Differentiate between usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP)

High resolution CT appearance

  • Usual interstitial pneumonia:
    • Apico-basal gradient
    • Heterogeneous
    • Honeycombing
    • Traction bronchiectasis
  • Non-specific interstitial pneumonia:
    • No gradient
    • Homogeneous
    • Ground-glass opacity
    • Micronodules

3

State two important aspects of Hx to diagnose ILD

  • Occupational Hx
  • Environmental Hx

4

How can the causes of interstitial lung disease be categorised?

  • With known cause
  • Associated with systemic disorders
  • Idiopathic

5

Name three interstitial lung diseases with known cause

  • Occupational
    • Asbestosis; coalworker's pneumoconiosis; byssinosis (cotton, hemp, flax); silicosis; berylliosis
  • Drugs
    • eg. Nitrofurantoin; bleomycin; amiodarone; sulfasalazine
  • Extrinsic Allergic Alveolitis
  • Infections
    • TB; viral; fungal

6

What is Extrinsic Allergic Alveolitis (EAA)?

Hypersensitivity reaction in sensitised individuals following inhalation of organic allergens eg. dust; mould; proteins

Includes:

  • Farmer's lung
  • Pigeon-fancier's lung
  • Malt worker's lung
  • Sugar worker's lung

7

Differentiate acute and chronic extrinsic allergic alveolitis

  • Acute: acute inflammatory cells infiltrate alveoli
    • Fever, rigors
    • Dry cough, dyspnoea, crackles
  • Chronic: granuloma formation and obliterative bronchiolitis
    • Increase exertional dyspnoea
    • Weight loss
    • T1RF, cor pulmonale

8

Outline the treatement of extrinsic allergic alveolitis

  • Acute: may spontaneously settle within 3/7
    • Remove allergens
    • 35-60% Oxygen
    • 40mg Prednisolone
  • Chronic:
    • Avoid allergens
    • Long term steroids

9

Name three interstitial lung diseases associated with systemic disorders

  • Sarcoidosis
  • Rheumatoid arthritis
  • SLE
  • Systemic sclerosis
  • Sjogren's syndrome
  • Ulcerative colitis
  • Autoimmune thyroid disease

10

What is sarcoidosis?

  • Multisystem inflammatory disease of unknown cause
  • Non-caseating granulomas
  • Associated with HLA-DRB1 and DQB1

11

Give three presenting features of sarcoidosis

Asymptomatic: most commonly an incidental finding

  • Dyspnoea
  • Dry cough
  • Malaise; weight loss
  • Hypercalcaemia
  • Neurosarcoid eg. facial nerve palsy

12

How does acute sarcoidosis present?

Löfgren's syndrome:

  • Bilateral hilar lymphadenopathy
  • Polyarthlagia
  • Erythema nodosum
  • Fever

13

Request three investigations for sarcoidosis

  • Pulmonary function tests
  • CXR
  • U+Es; serum ACE; serum Ca2+
    • ACE and Ca2+ produced by granulomas
  • Urinary Ca2+
  • ECG, 24h tape, echo, cardiac MRI
  • CT/MRI head

14

What chest x-ray findings are seen with sarcoidosis?

Siltzbach classification (0-4):

  • 0: Normal CXR
  • 1: Hilar/mediastinal lymphadenopathy
  • 2: Lymphadenopathy + parenchymal infiltration
  • 3: Parenchymal infiltration only
  • 4: Pulmonary fibrosis: upper zones, fissures, and nodules

15

What are the PFT results for the stages of sarcoidosis?

  • Stage 0-3: obstructive
  • Stage 4: restrictive

16

Name three causes of bilateral hilar lymphadenopathy

  • Sarcoidosis
  • Infection: eg. TB, mycoplasma
  • Malignancy
  • Organic dust disease: EAA; occupational asthma
  • Langerhans cell histiocytosis

17

Outline the treatment of sarcoidosis

60% will self-resolve in 2 yrs

  • Acute: bedrest and high-dose prednisolone
  • IV methylprednisolone or immunosuppressants, if any of:
    • Symptomatic with CXR stage 2 or 3
    • Hypercalcaemia
    • Heart or neuro involvement

18

Outline the diagnostic criteria for idiopathic pulmonary fibrosis

All of the following:

  • Exclusion of other known causes of ILD
  • Presence of a definite UIP pattern on HRCT prior to biopsy
  • Specific combination of HRCT and lung biopsy pattern
  • Pulmonary function tests: restrictive pattern + impaired TLCO
  • Bx must not show any features to support an alternative Dx.

19

How does idiopathic pulmonary fibrosis present?

Typically aged over 45

  • Progressive/persistent exertional dyspnoea
  • Persistent dry paroxysmal cough
  • Bibasal fine end-inspiratory crepitations
  • Clubbing
  • Abnormal CXR/CT
  • Reduced TLCO and restrictive pattern on spirometry

20

What is the hallmark of IPF?

Absence of inflammation

21

Outline the management of idiopathic pulmonary fibrosis

Aim to slow progression of pulmonary fibrosis

Life expectancy is 3-4y

  • Pulmonary rehabilitation
  • Smoking cessation
  • End of life care; DNACPR; ReSPECT form
  • Supportive care
    • Supplementary oxygen
    • Consider benzodiazepines/opioids for breathlessness
  • Consider anti-fibrotics eg. pirfenidone; nintedanib
  • Lung transplant