Interstitial Lung Disease Flashcards

Question

Lung volumes, gas transfer and walking tests in IPF

Answer 1

-Lung volumes are reduced in IPF -TCO and KCO are reduced in IPF (the TCO tends to fall before the KCO) -Patients with IPF (and others ILDs) tend to experience oxygen desaturation on exercise / walk tests

Answer 2

-Reticular pattern which is made of intra-lobular lines -Traction bronchiectasis / bronchiolectasis -Honeycomb cyst formation -These changes must be subpleural and basally dominant 1, First changes in phrenic angles 2. Shaggy hemidiaphragm and heart border

Answer 3

-Possible UIP pattern =Consider risk vs benefit =2% risk of death

Answer 4

-Risk depends on patients co-morbidities -Overall 30-day mortality around 1-2% and morbidity 10% -Risk is higher in older patients with more extensive fibrosis disease on CT and poorer lung function

Answer 5

-Idiopathic pulmonary fibrosis (IPF) progressive disease with a median survival of 3 to 5 years following diagnosis -Only 20% survive longer than 5 years -IPF shortens life expectancy by around 7-9 years -IPF has a lower survival rate than many cancers

Answer 6

-Pirfenidone -Nintedanib =Slows rate of decline in FVC by 50%

Answer 7

Inhibitor of multiple tyrosine-kinases -Inhibit growth factors in lung

Answer 8

-Nausea -Moderate rash/ photosensitivity -Dyspepsia -Weight loss -20% discontinuation

Answer 9

-Nausea -Diarrhoea -Dyspepsia -20% discontinuation

Answer 10

-Smoking cessation -Treat co-existing acid reflux symptoms (? micro aspiration of gastric contents) -Ambulatory and domiciliary oxygen -Pulmonary rehabilitation – proven and cost effective in IPF -Lung transplantation – only suitable for younger patients without comorbidities

Answer 11

Systemic disease of unknown aetiology characterised by the presence of non-caseating granulomas

Answer 12

-Peak at 25-35 and 50-60 -Does not show in elderly -More common in temperate climates

Answer 13

-Skin – rash of various types -Eyes - iritis -Joints – arthralgia -Neurological – facial nerve palsy -Liver –cirrhosis

Answer 14

-Fatigue -Symptoms of hypercalcaemia (polyuria, polydypsia, renal stones, constipation, confusion) -Enlarged lymph nodes -Night sweats

Answer 15

-Cough- almost always dry (granulomas affecting bronchi) -Breathlessness

Answer 16

-May be evidence of systemic disease (skin, eyes, splenomegaly, palpable lymphadenopathy) -Chest examination is often normal in sarcoidosis. -Crackles are infrequent - even with extensive abnormalities are present on CXR

Answer 17

-Based on CXR appearance 1. Bilateral Hilar Lymphadenopathy (BHL) with normal lung parenchyma, erythema nodosum 2. BHL with lung infiltrates 3. Lung infiltrates without hilar node enlargement on CXR

Answer 18

-Will be normal in stage 1 disease or mild stage 2 or 3 disease -Usually, a restrictive defect with more extensive stage 2 or 3 disease -Occasionally an obstructive defect is seen with sarcoidosis, implying endobronchial disease

Answer 19

-Be aware that sarcoidosis is a cause of hypercalcaemia -Serum angiotensin converting enzyme (SACE) may be raised in sarcoidosis. This test is not specific for sarcoidosis but is sometimes used to monitor disease activity

Answer 20

-Patients with sarcoidosis exhibit ‘anergy’ characterised by a blunted T-cell response -This can be demonstrated using the tuberculin skin test – it is usually negative in patients with sarcoidosis

Answer 21

-Stage 1 sarcoidosis with erythema nodosum often does not need histological proof -A confident diagnosis of Stage 2 or 3 sarcoidosis may require a biopsy -Biopsy the easiest site e.g. skin, lymph nodes if enlarged -In the lung, a transbronchial biopsy will be positive in 80% of cases -Sometimes a surgical lung biopsy is needed

Answer 22

-Stage 1 sarcoidosis spontaneously remits in 60% of patients within 1-2 years, so hardly ever warrants treatment -Stage 2 and 3 sarcoidosis can spontaneously remit, or does not progress, in 20-40% of cases, so should only be treated if there is evidence of active progressive lung disease or chest symptoms -Failure to treat progressive stage 2 or 3 sarcoidosis can lead to permanent lung scarring with irreversible loss of function (sometimes called stage 4 disease)

Answer 23

-Corticosteroids are the standard treatment for sarcoidosis -No randomised controlled trials have been performed in pulmonary sarcoidosis -Over 50 years of observational data shows that corticosteroids can be associated with resolving CXR appearances and improved lung function tests and symptoms

Answer 24

1. Initial control 2-4 weeks 40mg prednisolone 2. Taper 2-4 months down from 40 to 5mg prednisolone 3. Maintenance 3-9 months at 5mg prednisolone 4. Taper by 1mg/month 5. Observation period -If relapse, restart steroids -Total period typically 18-24 months