Lung disease group work Flashcards
malignant mesothelioma
a tumour of mesothelial cells usually occurs in the pleura rare in the peritoneum or other organs ass/w. occupational exposure to asbestos 90% will have previous asbestos exposure on 20% will have pulmonary asbestosis latent period between exposure and tumour presentation can be up to 45yrs
clinical features: chest pain dyspnoea weight loss clubbing recurrent pleural effusions signs of mets
poor prognosis eg<2years without pemetrexed chemo
asbestosis
caused by the inhalation of asbestos fibres
degree of exposure is related to the degree of fibrosis
similar features to other fibrotic lung disease, progressive dyspnoea, finger clubbing, fine end-inspiratory crackles
causes pleural plaques and increases the risk of bronchial adenocarcinoma and mesothelioma
management is symptomatic
coal workers pneumoconiosis (CWP)
common dust disease in areas with underground coal mines
results from inhalation of coal dust particles over 15-20yrs
dust particles are ingested by macrophages, which release enzymes leading to lung fibrosis
clinically asymptomatic, but co-existing chronic bronchitis is common
CXR: many round opacities (1-10mm), especially in the upper zones
management: avoid exposure to coal dust, treat chronic bronchitis
progressive massive fibrosis
due to progression of CWP
causes progressive dyspnoea, fibrosis, and eventually cor pulmonale
CXR: upper zone fibrotic masses (1-10cm)
caplan’s syndrome
the association between RA, pneumoconiosis, and pulmonary rheumatoid nodules
silicosis
inhalation of silica particles
clinical features: progressive dyspnoea, increased incidence of TB, CXR diffuse miliary or nodular pattern in upper or mid zones, egg shell calcification of the hilar nodes
spirometry: restrictive ventilatory defect
idiopathic pulmonary fibrosis (IPF)
interstitial pneumonia
inflammatory cell infiltrate and pulmonary fibrosis of unknown cause
commonest cause of interstitial lung disease
Sx: dry cough, exertional dyspnoea, malaise, weight loss, arthralgia
Signs: cyanosis, clubbing, fine end-inspiratory crackles
complications: respiratory failure, increased risk of lung cancer
management: best supportive care, O2, rehab, opiates, palliation
do NOT use high dose steroids unless diagnosis is in doubt
consider patients for clinical trials or transplantation
prognosis: 50% 5yr survival rate
interstitial lung disease (ILD)
conditions that generally affect the lung parenchyma in a diffuse manner
chronic inflammation and/or progressive interstitial fibrosis
clinical: dyspnoea on exertion, non-productive paroxysmal cough, abnormal breath sounds, restrictive pulmonary spirometry with decreased CO diffusing capacity
pathological: fibrosis and remodelling of the interstitium; chronic inflammation; hyperplasia and type II epithelial cells or type II pneumocytes
classification:
known cause - occupational, drugs, hypersensitivity reactions, infections, gastro-oesophageal reflux
systemic associations - sarcoidosis, RA, SLE, UC
idiopathic - IPF
extrinsic allergic alveolitis
in sensitized individuals, inhalation of allergens provokes a hypersensitivity reaction
in the acute phase, the alveoli are infiltrated with acute inflammatory cells. the airways constrict and air gets ‘trapped’ leading to air pockets in the alveoli
in chronic, granulomas can form along with obliterative bronchiolitis
causes: bird-/pigeon-fancier’s lung, farmer’s lung
clinical:
4-6h post exposure: fever, rigors, myalgia, dry cough, dyspnoea, crackles (no wheeze)
chronic: increasing dyspnoea, weight loss, exertional dyspnoea, type 1 respiratory failure, cor pulmonale
management: remove allergen, give O2, then oral prednisolone (40mg/24h) before reducing the dose
chronic: avoid exposure to allergens, wear a facemask or +ive pressure helmet. long term steroids often achieve improvement
sarcoidosis
multisystem granulomatous disease of unknown cause
usually affects adults 20-40yrs
more common in women
ass/w. HLA-DRB1 and DQB1 allelles
clinical: 20-40% incidental finding after routine CXR and is thus asymptomatic
acute sarcoidosis often presents with erythema nodosum (shins) +/- arthralgia. usually resolves spontaneously
pulmonary disease:
90% have abnormal CXRs with bilateral hilar lymphadenopathy +/- pulmonary infiltrates or fibrosis
Sx: dry cough, progressive dyspnoea, decreased exercise tolerance and chest pain. Sx progress in 10-20% with concurrent deterioration in lung function
non-pulmonary signs are legion:
lymphadenopathy, hepatomegaly, splenomegaly, uveitis, conjunctivitis, keratoconjunctivitis sicca etc etc
tests:
lavage shows increased lymphocytes in active disease, neutrophils with pulmonary fibrosis
bone x-rays show punched out lesions in terminal phalanges
for acute treat with bed rest and NSAIDs.
bilateral hilar lymphadenopathy will often resolve spontaneously
prognosis: 60% with thoracic sarcoidosis resolve over 2 years. 20% respond to steroid therapy - therapy required for the rest
management of chronic asthma
step 1: occasional SABA PRN. if used more than twice daily, or nighttime Sx, go to step 2
2: standard dose inhaled steroid eg beclametasone
3: add a LABA (salmeterol) and increase dose of steroid if control still not achieved. if no effect stop LABA and review2 diagnosis
4: leukotriene antagonist
5: add regular oral prednisolone
