Interstitial lung disease Flashcards
Langerhans histiocytosis (AKA histiocytosis X).
a) What is it?
b) Risk factors
c) Presentation
d) Treatment
a) - Over-production of Langerhan cells (histiocytes), that results in granuloma formation
- May affect several organs (e.g. skin, lung, LNs, liver)
- Some experts say it is a cancer
- Child onset more likely to be multisystem, adults more likely to get lung LCH
b) - BRAF gene
- Smoking
c) - Lung - cough, SOB, recurrent pneumothorax, granulomas
- Skin - rash
- other - e.g. cranial DI
d) - Surgery
- Chemoradiotherapy
Extrinsic allergic alveolitis
a) 3 forms and onset
b) Test results
c) Management
a) Acute: fever, cough, SOB that occur 4-6h post exposure
Subacute: associated with weight loss and fatigue
Chronic: progressive SOB, upper lobe fibrosis
b) IgG precipitins positive
- Negative for “allergic” features (e.g. skin prick, eosinophils, IgE)
c) - Avoidance of exposure
- Steroids if acute severe symptoms
IPF
a) 4Ds
b) Management
a) Dry cough
Dyspnoea
Digital clubbing
Diffuse basal end-inspiratory crackles
b) - Smoking cessation, pulmonary rehab, oxygen therapy
- Pirfenidone for mild-moderate IPF - slows down progression as antifibrotic/anti-inflammatory
- PPI if reflux symptoms
- Lung transplant - younger, less comorbid patients - indication is TLCO <40%, or drop in FVC 15% or TLCO 10% over 6 months
- “Triple therapy”: Oral prednisolone, oral azathoprine, NAC
Asbestosis
a) vs. EEA or coal-worker’s lung
b) Diagnosis
c) Treatment
d) Occupations at risk
a) - Asbestosis predominantly affects the lower lobes
- EEA and coal-workers lungs affects upper lobes predominantly
b) - HRCT
(note: asbestos bodies in BAL are not diagnostic as they may be present in those without asbestosis)
c) - Avoidance of further exposure
d) Plumbers, builders, boiler fitters, etc.
Rheumatological disease - most common form of ILD
Non-specific interstitial pneumonitis (NSIP)
NSIP
Connective tissue disease
Drugs - amiodarone, MTX, nitrofurantoin
Lymphangeoleiomyomatosis (LAM)
a) Risk factors
b) Clinical features
c) Investigation findings
d) Management
a) - May be sporadic or associated with TB
- Commonly have angiomyolipomas (AML)
- VEG-D positive
- Most common in women aged 30-50s
b) - Present with recurrent pneumothorax and dyspnoea
- Also get chylous effusions
- Develop ILD
c) - HRCT shows thin-walled cysts, ground glass changes
d) Conservative: sirolimus, progesterone
Definitive: lung transplant
Pulmonary sarcoidosis stages
0 normal
1 BHL
2 BHL + infiltrates
3 Pulmonary infiltrates without BHL
4 Pulmonary fibrosis
Higher stages have reduced rates of spontaneous remission
Serum ACE raised
Sarcoidosis
Lymphoma
TB
Asbestosis
Silicosis
Gauchers
Bronchiolitis obliterans
a) causes
b) features
c) CT findings
a) RA
b) Obstructive picture
c) Bronchiolar thickening and air trapping
Elastic recoil vs compliance
ILD - high elastic recoil, low compliance
Emphysema - low elastic recoil, high compliance
Lower compliance in restrictive lung diseases like ARDS make ventilation very difficult as very high pressures are needed
Bronchiectasis
a) Subtypes
a) Cylindrical - often reversible
- Varicose
- Cystic
b)
Causes of lung fibrosis
a) Upper zone predominant
b) Lower zone predominant
c) Mixed upper/lower zones
d) Signs on CXR
a) TB, sarcoid, Ank Spond, radiotherapy, EAA, progressive massive fibrosis, coal workers pneumoconiosis
b) Scleroderma, RA, drugs (amiodarone), chronic aspiration (more so RLZ), asbestosis, carcinomatosis lymphangitis
c) CF, histiocytosis, pneumoconiosis, NSIP, UIP, lymphangiomyomatosis, tuberous sclerosis, neurofibromatosis
d) Reticular markings +/- nodules +/- volume loss (e.g. elevated hilum in upper lobe fibrosis)
90 year old man presents with gradual increasing SOB. On examination, he has a photosensitive rash on his face, is in AF, and has bilateral fine crepitations. TSH is slightly high on blood testing.
a) Cause
b) Other possible features
a) Amiodarine
b) Corneal deposits
Metallic taste
Peripheral neuropathy, ataxia
Hepatitis
Arrhythmias eg torsades
