Pulmonary vasculitides, eosinophilia Flashcards
What are cardiac causes of clubbing
Cyanotic HD
IE
Atrial myxoma
What are respiratory causes of clubbing
Lung cancer
TB
Asbestos
Fibrosing alveolitis
CF
Bronchiectasi
Abscess
Empyema
What are other causes of clubbing
Chron’s
Cirrhosis
Grave’s
Whipples
What causes respiratory acidosis
COPD Asthma Pulmonary oedema Neuromuscular Obesity HYpoventilation Benzo / opiate overdose
What causes respiratory alkalosis
Anxiety Hyperventilation PE Salciclyte SAH Encephalitis Stroke Altitude Pregnancy
What is granulomatous polyangiitis
formerly known as Wegener’s granulomatosis.
Necrotizing granulomatous vasculitis affecting upper and lower respiratory tract + kidney
Features
* upper respiratory tract: epistaxis, sinusitis, nasal crusting
* lower respiratory tract: dyspnoea, haemoptysis
* rapidly progressive glomerulonephritis (‘pauci-immune’, 80% of patients)
* saddle-shape nose deformity
* also: vasculitic rash, eye involvement (e.g. proptosis), cranial nerve lesions
Granulomatosis with polyangiitis
How do you investigate
- cANCA positive in > 90%, pANCA positive in 25%
- chest x-ray: cavitating nodules or lesions > CT-PET showing increased uptake > CT guided bx: fibroinoid necrosis
- renal biopsy: epithelial crescents in Bowman’s capsule
Management of Granulomatosis with polyangiitis [4]
- CCS - high-dose methyl- prednisolone
- pulsed IV cyclophosphamide
- Plasma exchange- used for renal disease but its role in pulmonary disease is less clear.
- Remission can be sustained with less aggressive agents, such as azathioprine. Methotrexate can be used as an alternative in less severe renal disease.
What is Churg Straus
Eosinophilic vasculitis
3 stages
Stage 1 =. allergy
Asthma
Sinusitis / rhinitis
Polyps
Stage 2 = eosinophilia
Stage 3 = vasculitis
Heart, lung, GI, kidney
What is Kartamenger syndrome
Primary ciliary dyskinesia
What is suggestive of Katmenger syndrome
Sinusitis
Bronchiectasis
Dextrocardia
Subfertility
What causes altitude sickness
Chronic hyperbaric hypoxia at high altitude
Altitude sickness
How do you prevent and treat
No >500m per day
Acetazolamide
Descent
What can altitude sickness progress too
Pulmonary oedema
Cerebral oedema
Altitude sickness
What does cerebral oedema present with
Headache
Ataxia
Papilloedema
Altitude sickness
How do you treat
Descent
Dexamethasone
Goodpasture’s syndrome
Features, investigation finding
Causes alveolar haemorrhage resulting from anti-glomerular basement membrane (GBM) antibodies that can also cause crescentic glomerulonephritis. Features: pulmonary haemmorrhage
Investigation: renal biopsy shows linear IgG deposits along basement membrane
Pulmonary eosinophilia are a heterogenous group of diseases
Pulmonary eosinophilia causes
associated with eosinophilic alveolar infiltrates and peripheral eosinop
- Parasitaemia, e.g. helminthic infections.
- Resolving pneumonia.
- Hydatid disease.
- Sarcoidosis.
- Polyarteritis nodosa.
- ABPA.
- EGPA.
- Acute and chronic eosinophilic pneumonia.
How would pulmonary eosinophilia typically present?
- Severe haemoptysis is recognized, but is often absent.
- Cough, dyspnoea, pleuritic pain.
- Clinical examination may reveal evidence of underlying extra thoracic and thoracic disease, e.g. purpuric rash, joint pain/inflammation.
- Pulmonary vasculitis should be considered in any persistent, aggressive or unusual presentation of pneumonia.
Contents [4]
Coal Workers Pneumoconiosis (CWP)
Asbestosis
Silicosis
Mesothelioma
CWP
Ep
Ax [2]
Pathophysiology [3]
Ep: countries w/ underground coal mines Ax: inhalation of coal dust particles, over 15-20y Px: - dust particles of 1 to 3 micrometres - ingested by macrophages which die - releasing enzymes and causing fibrosis
CWP
Presentation [2]
Investigations
Asymptomatic
Can co-exist with chronic bronchitis
Ix:
CXR, PFT
CWP
Describe CXR
Describe PFT
CXR:
- Multiple upper zone round opacities
- 5-10mm
PFT: restrictive pattern
CWP Mx [3]
Complication
- Avoid coal dust
- Treat co-existent chronic bronchitis
- Claim compensation
Complication: Progressive massive fibrosis
Progressive massive fibrosis [5]
Progressive SOB
Worsening fibrosis
Eventually developing cor pulmonale
CXR showing upper AND middle zone fibrotic masses
- start from periphery extending to hilum
Silicosis
Epidemiology [4]
Ax
Presentation [2]
Ep:
- Metal mining
- Quarrying
- Sand blasting
- Pottery/ceramic manufacture
Ax: inhalation of silica dust particles
Presentation
- Progressive SOB
- Increased TB incidence in these patients
Silicosis CXR [3] PFT [1] Mx [2] Complication [1]
CXR
- Diffuse miliary or nodular pattern
- In upper and mid zones
- Egg shell calcification of hilar nodes
PFT: restrictive
Mx: avoid silica, claim compensation
CX: PMF
Asbestosis
Epidemiology
Aetiology
What are the different types of asbestos fibers [4]
Ep: building trade (fire proofing, pipe lagging, electrical wire insulation, roofing felt)
Ax:
- inhalation of asbestos fibres:
- crocidolite (blue) = most fibrogenic
- chrysotile (white) = least fibrogenic
- amosite = intermediate fibrogenicity (least common)
Asbestosis Onset [1] Symptoms [1] Signs [2] Pathophysiology [2] Incidence
Symptoms - onset >10y post exposure - progressive SOB Signs - finger clubbing - fine ended crackles
Pathophys:
- Particles deposited at alveolar duct bifurcations
- Cause alveolar macrophage alveolitis
Inc: 90% patients have asbestos exposure but only 20% have had asbestosis
Asbestosis
Investigation [2]
Mx [4]
Cx [2]
Ix:
- CXR or HRCT thorax
- PFT
Mx (symptomatic):
- Antibiotics (if infection)
- Bronchodilators if obstructive
- Smoking cessation
- Claim compensation
Cx: cor pulmonale, malignancy
What are the cancers related to general asbestos exposure? [4]
What are benign asbestos related diseases [3]
Malignant: lung cancer mesothelioma colon cancer laryngeal cancer
Benign:
Pleural plaques
Diffuse pleural thickening
Benign asbestos related pleural diffusion
Asbestosis - what can you see on CXR or HRCT? [3]
What can you see on PFT [2]
- Lower zone linear interstitial fibrosis
- Progressively involving entire lung
- Pleural thickening
PFT:
- Restrictive
- But can be obstructive esp if smoker
Mesothelioma
Ax
Px [2]
Presentation [4]
Ax: inhalation of asbestos fibres Px: - tumour of mesothelial cells of pleura - or more rarely peritoneum Presentation: - Chest pain, SOB - Weight loss, fever - Clubbing - Recurrent pleural effusions
Mesothelioma: presentation of metastases [4]
- Lymphadenopathy
- Hepatomegaly
- Abdo pain
- GI obstruction
Mesothelioma investigations
initial [2]
dx [2]
Initial:
- CXR/CT (pleural thickening)
- Pleural aspiration (blood)
Diagnostic:
- USS or CT guided percutaneous pleural biopsy
- Thoracoscopy under LA
Why is thoracoscopy both therapeutic and diagnostic
Can obtain pleural biopsy
- Pleural fluid drainage
- Pleurodesis
Mesothelioma staging [3]
CT thorax
Mediastinoscopy
Video-assisted thoracoscopy
TMN staging mesothelioma: stage 1 [2]
T1N0M0
T1a - limited to ipsilateral parietal pleura
T1b - ipsilateral parietal pleura + visceral pleura
TMN staging mesothelioma: stage 2 [4]
T2N0M0 -1a or 1b \+ confluent diaphragm or \+ visceral pleura or \+ lung involvement
TMN staging mesothelioma: stage 3
T3M0
T1-3N1M0
T1-3N2M0
T3M0
- locally advanced tumour
T1-3N1M0
- ipsilateral, pulmonary or hilar LN
T1-3N2M0
- Sub carinal or ipsilateral mediastinal LN
Stage 4 mesothelioma
Any T4:
- Locally advanced technically irresectable tumour
Any N3
- Contralateral mediastinal, LN or
- internal mammary and ipsilateral/contralat supraclavicular LN
Any M1 (distant mets)
Mesothelioma management [2]
Prognosis
Chemotherapy - pemitrexed and cisplatin
Pleurodesis and in-dwelling pleural drain
Prog: <2y
