restrictive disease

Question 1

What is Interstitial lung disease (ILD) ? ix+dx? Mx?

Answer 1

Term for conditions that causes inflammation and fibrosis (replacement of tissue with scar tissue) of lung parenchyma. There is a reduction in elasticity.

Types of Fibrosis

• IPF
• Drug induced: methotrexate, nitrofurantoin, amiodarone
• A1AD, RA, SLE
• Hypersensitivity Pneumonitis (HP)/ Extrinsic allergic alveolitis (EAA)/ bird fancier/farmers lung/ malt worker

Dx:

(1. ) Spirometry: normal FEV1/FVC
(2. ) Reduced TCO (gas transfer CO)
(3. ) High resolution CT = GGO
(4. ) Lung biopsy, not often done as can worsen condition

Mx:

(1. ) Remove or Rx underlying cause
(2. ) Stop smoking
(3. ) Pulmonary rehabilitation
(4. ) Home oxygen if hypoxic at rest
(5. ) Pneumococcal + flu vaccine
(6. ) Advanced care planning and palliative care if appropriate
(7. ) Lung transplant

Question 2

What is IPF? Presentation?Mx?

Answer 2

Progressive fibrosis with no clear cause. 2-5yr prognosis from dx.

Syx

• Asyx
• Progressive sob
• Dry cough
• o/e: bibasal fine inspiratory crackles, finger clubbing

Mx

(1. ) Pulmonary rehab
(2. ) Medication slows down progress of disease:
- Pirfenidone
- Nintedanib

Question 3

Pneumoconiosis : what is it? types? presentation ?

Answer 3

• Permanent alteration of lung structure due to inhalation of mineral dust
• Inhaled particles accumulate around bronchioles and produce localised scarring. In some cases it can produce a blackening of the lung tissues (anthracosis).
• Most important pneumonoconiosis = coal worker’s, silicosis, asbestosis

Asbestosis

• lung fibrosis related to the inhalation of asbestos
• it can cause: lung fibrosis, pleural thickening, adenocarcinoma, mesothelioma

presentation
o progressive dyspnoea
o finger clubbing
o fine inspiratory crackles

Question 4

Sarcoidosis Syx

Answer 4

Multisystem disorder of unknown aetiology characterised by non-caseating granulomas
RF: young, African, women

Syx

• *MCQ: 20-40y Africa female with dry cough + sob + nodules on shins**
  (1. ) Chest syx:
• Dyspnoea
• Non-productive cough
• Pulmonary fibrosis
• Mediastinal lymphadenopathy
  (2. ) Skin:
• Erythema nodosum (tender red nodules on shin)
• lupus pernio (blueish-red nodules over nose/cheeks/ ears)
  (3. ) Systemic syx: fatigue, malaise, fever
  (4. ) Liver: nodules, cirrhosis, cholestasis
  (5. ) Polyarthralgia (pain in multiple joints)

Question 5

Sarcoidosis: IX + DX, MX

Answer 5

Ix

(1. ) Bloods:
- raised Ca, CRP, ACE, Ig, soluble IL2-R
- UE, LFT
(2. ) Urine dip
(3. ) ECG
(4. ) Imaging:
- CXR: b/l hilar lymphadenopathy
- CT
- MRI
- PET Scan: active areas of inflammation

(5.) Biopsy + histology (GOLD) via skin or bronchoscopy: ‘no-caseating granuloma with epithelioid cell’

Mx
Some people it resolves on its own in 6m.
(1.) No Rx if no/mild syx
(2.) PO steroid for 6-24m (+bisphosphonates)
(3.) Methotrexate or azathioprine
(4.) Lung transplant