sarcoidosis, fibrosis, bronchiectasis and EAA Flashcards
what is saroidosis?
a chronic granulomatous disorder that affects the lung, skin and eyes. It is characterised by accumulation of lymphocytes and marcophages in the form of non-caseating granulomas in the lungs and other organs. Lung and LN’s involved in over 90%.
whats the S&S of sarcoidosis?
SOB on exertion cough chest pain haemoptysis fever fatigue night sweats uveitis - red painful eye with blurred vision
what are the stages of sarcoidosis
Based on CXR findings
stage 1 = bilateral hilar lymphadenopathy
stage 2 = bilateral hilar lymphadenopathy + pulmonary filtrates
stage 3 = pulmonary infiltrates without hilar lymphadenopathy
stage 4 = extensive fibrosis with honeycombing
stages do not indicate severity
what do investigations for sarcoidosis show?
CXR
-staging
FBC, U&E’s, LFTS, creatinine
- transaminits
- anaemia and leucopenia
- hypercalcaemia
bronchoalveolar lavage
- BAL lymphocytosis
Skin biopsy
- non caseating granulomas
what is pulmonary fibrosis?
a rare, chronic, life threatening disease that manifests over several years and is characterised by the formation of scar tissue within the lungs and progressive dyspnoea
what are the causes of pulmonary fibrosis?
25% idiopathic
occupational/environmental - silicosis, asbestosis
Drugs - MTX, amiodarone, nitrofurantoin
what are S&S of IPF?
dyspnoea dry hacking cough crackles (snow or velcro) weight loss and fatigue clubbing cyanosis
what InV are done in IPF?
CXR
- bibasilar, peripheral and bilateral asymmetical reticular opacities
high resolution CT
- subpleural honeycombing
Immunoglobulin
- ANA (30%) and RF (10%)
Pulmonary function tests
- restrictive pattern
what is bronchiectasis?
Chronic infection of the bronchi and bronchioles leading to permanent dilatation of these airways.
what pathogens are implicated in bronchiectasis?
H. influenzae
Strep. pneumoniae
Staph. aureus
Pseudomonas aeruginosa.
what are the causes of bronchiectasis?
Congenital
- cystic fibrosis
- Young’s syndrome
- primary ciliary dyskinesia
- Kartagener’s syndrome
Post-infection
- measles; pertussis; bronchiolitis; pneumonia; tb; hiv.
Other
- bronchial obstruction (tumour, foreign body); allergic bronchopulmonary aspergillosis hypogammaglobulinaemia; rheumatoid arthritis; ulcerative colitis; idiopathic.
what are the S&S of bronchiectasis?
persistent cough
copious purulent sputum
intermittent haemoptysis.
finger clubbing
coarse inspiratory crepitations
wheeze
what InV are done in bronchiectasis ?
Sputum culture
CXR
- cystic shadows, thickened bronchial walls (tramline and ring shadows)
hrct chest
- to assess extent and distribution of disease.
Spirometry often shows an obstructive pattern; reversibility should be assessed.
Bronchoscopy
- to locate site of haemoptysis, exclude obstruction and obtain samples for culture.
Other tests: serum immunoglobulins; cf sweat test; Aspergillus precipitins or skin-prick test.
what is extrinsic allergic alveolitis?
t-cell mediates hypersensitivity reaction to inhaled allergen
in the acute phase alveoli are infiltrated with acute inflammatory cells
in chronic exposure, granuloma formation and obliterative bronchiolitis occur
what are the typical allergens causing EAA?
mould - farmer and malt workers lung
sawdust - saw mill workers lung
spores - mushroom workers lung
protein in bird shit - bird fanciers lung
