Pulmonary Fibrosis + Bronchiectasis Flashcards
Types of PF
Replacement fibrosis = secondary to lung damage
Focal fibrosis = response to irritants
Idiopathic PF = can cause diffuse parenchymal lung disease
Pathology of IPF
Excess collagen in lung
Type 1 alveoli = squamous cells, form near continuous barrier between air + connective tissue
Type 2 alveoli = have microvilli, secrete surfactant, can divide + become type 1 or type 2
Between type 1 + type 2 alveoli is interstitial tissue containing macrophages + fibroblasts
When alveoli is damaged, type 1 cells release TGFB1, stimulated fibroblasts to differentiate into myofibroblasts -> these produce reticular + elastic fibres
Type 2 cells over-proliferate in fibrosis, myofibroblasts don’t undergo apoptosis
Thickened interstitial layer -> causes problems with ventilation + oxygenation (restricted lung expansion)
Decreased total lung capacity
Decreased forced vital capacity
Decreased FEV1
Loss of alveoli causes fluid filled cysts (honeycombing)
RF for PF
Smoking
Bird + animal droppings
Herpes virus
GORD
Fam hx
Radiation
Drugs - chemo, amiodarone, methotrexate, nitrofurantoin
What other conditions is PF associated with?
Thyroid disease
Systemic sclerosis
RA
SLE
Autoimmune liver disease
Investigation results for PF
Low O2, CO2 retention if severe I
ncreased CRP
CXR: decreased lung volume, bilateral lower zone reticulonodular shadows, honeycomb lung
CT is essential for diagnosis
Spirometry = decreased FVC, FEV1/FVC ration equal or increased
Complications of PF
Respiratory failure, increased risk of lung cancer
Management of PF
Pirfenidone or nintedanib
Supportive therapy
Lung transplant
Causes of bronchiectasis
Post infection
No known cause
RF for bronchiectasis
CF, Kartageners TB, pertussis, measles, pneumonia
Bronchial obstruction, aspergillosis, hypogammaglobulinaemia, gastric aspiration
S+S of bronchiectasis
Winter exacerbation - fever, cough, sputum, pleuritic chest pain + SOB
Haemoptysis + wheeze
Clubbing, low pitched crackles + wheeze
Investigations for bronchiectasis
CXR
Sputum MC+S
Spirometry (reversible obstruction)
CT for diagnosis
Treatment of bronchiectasis
physio, abx, bronchodilators
What are common causes of pneumonia + S+S of each
Staph = following flu
H influenza = if COPD L
egionella = dry cough, hyponatraemia, lymphopenia
What is sarcoidosis?
Causes constitutional upset eg weight loss, fever, night sweats
Shows restrictive pattern on spirometry
Caused by non-caseating epitheloid granuloma deposits
Causes lung + skin problems
Pathology of bronchiectasis
Permanent dilatation + thickening of airways associated with chronic cough, sputum production, bacterial colonisation + recurrent infection
Complications of bronchiectasis
Progressive loss of lung function
RHF secondary to chronic resp disease
Urinary incontinence + sexual dysfunction
Chronic tiredness
Nutritional deficiency
When can an acute infective exacerbation of bronchiectasis be diagnosed?
Acute deterioration with worsening cough +/- systemic upset
Presence of purulent sputum
Conditions leading to lung transplantation
COPD
CF
Idiopathic interstitial pneumonias
IPAH, Eisenmenger’s syndrome
Sarcoidosis
What are sicca symptoms?
Dry eyes + dry mouth
Associated with Sjogren’s syndrome
What are the types of intersitital lung disease?
Asbestosis
Sarcoidosis
IPF
RA associated
Drug induced
Pneumoconiosis
What changes do you get on CXR from interstitial lung disease?
Reticulodular changes = lines and dots from fibrosis
Volume loss
Specific distribution:
Upper lobe fibrosis (due to increased ventilation) = TB, hypersensitive pneumonitis, sarcoidosis
Lower lobe (perfuse more than you ventilate) = IPF, drugs
What CT changes + signs will be present in idiopathic pulmonary fibrosis?
IPF = UIP pattern on CT (honeycombing - basal, bilateral and subpleural, on the peripheries)
50% will have clubbing
Have fine late basal inspiration crackles (velcro crackles)
SOBOE + dry cough
What does the colour of sputum indicate?
Sputum is coloured due to neutrophils
What is procalcitonin?
Early marker of infection
How to reduce risk of exacerbation in bronchiectasis?
Physio, neb saline, azithromycin, nebulised abx (colisitin, tobramycin)
