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Respiratory > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

What is the interstitium?

A

-Space between the capillary endothelial cells and alveolar epithelial cells
=Collagen
=Fibroblasts
=Dendritic cells
=Where gas transfer occurs so thin

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2
Q

What is ILD?

A

-Diseases in which there is inflammation and/or fibrosis primarily to the lung interstitium

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3
Q

Classification of ILDs

A

-Known causes
-Idiopathic
-Rare/miscellaneous

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4
Q

Types of ILD with known cause or association

A

-Inhaled dusts/ antigens/ fibres
-Iatrogenic
-Connective tissue disease associated
-Smoking-related

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5
Q

Examples of haled dusts/ antigens/ fibres

A

-Coal Worker’s Pneumonconiosis
-Asbestosis
-Silicosis
-Hypersensitivity pneumonitis due to birds, moulds, other exposures

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6
Q

Examples of iatrogenic ILD

A

-Drugs
-Radiation-induced

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7
Q

Examples of connective tissue disease associated

A

-Rheumatoid
-Systemic Sclerosis
-Other CTD

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8
Q

Examples of smoking related ILDs

A

-Respiratory-bronchiolitis ILD (RB-ILD)
-Desquamative interstitial pneumonia (DIP)
-Langerhans cell histiocytosis

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9
Q

Examples of idiopathic IDLs

A

-Sarcoidosis
-Idiopathic Usual Interstitial Pneumonia (UIP) = Idiopathic pulmonary fibrosis (IPF)
- Idiopathic non-specific interstitial pneumonia (NSIP)
-Cryptogenic organising pneumonia (COP)
-Idiopathic Acute Interstitial Pneumonia (AIP)

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10
Q

Examples of miscellaneous and rare ILDs

A

-Eosinophilic pneumonia
-Lymphangioleiomyamotosis
-Alveolar Proteinosis
-Lipoid pneumonia
-Lymphocytic Interstitial Pneumonia

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11
Q

Most common ILDs

A

-Sarcoidosis
-Idiopathic Usual Interstitial Pneumonia/ Idiopathic pulmonary fibrosis

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12
Q

General presentation of ILD

A

-Breathlessness
-Cough
-Incidental findings on CXR or CT

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13
Q

General investigations in ILD

A

-HRCT scan (high resolution)
-Lung biopsy

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14
Q

Drugs often associated with ILD

A

-Amiodarone
-Methotrexate
-Leflunamide
-‘Biologicals’ e.g. anti-TNF, Rituximab
-Nitrofurantoin
-Sulphasalazine (anti-inflammatory)
-Bleomycin

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15
Q

Drugs rarely associated with ILD

A

-Omeprazole
-Statins
-SSRI anti-depressants

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16
Q

ILD examination findings

A

-Clubbing (IPF, Asbestosis)
-Features of connective tissue disease (RhA, systemic sclerosis)
-Lung crepitations (mid and late respiratory)

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17
Q

CXR in ILD

A

-Bilateral diffuse infiltrates
=Nodular
=Reticular
=Reticulo-nodular

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18
Q

Pathogenesis of IPF

A

-Normal injury to alveolar epithelial cells and response
-IPF
=Aberrant repair process= overactive inflammatory response so overactive fibroblasts= transdifferentiate into myofibroblasts so lots of collagen deposition (fibroblastic foci)
=Type 2 alveolar epithelial cells hypertrophy with abnormal phenotype and can’t differentiate into Type 1

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19
Q

Histology of IPF

A

-Subpleural dominant disease
-Grossly distorted lung architecture
-Honeycomb fibrosis/ cysts
-Temporal and spatial heterogeneity (fibrotic lung and inflamed lung closely adjacent to normal lung)
-Fibroblastic foci

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20
Q

Epidemiology of IPF

A

-Occurs world-wide
-5-15 cases per 100,000 population
-4-5000 new cases per year in UK
-Lothian approx 90 new cases per year
-Median age at presentation approx 70 yr
-M:F 2:1
-Smokers: non-smokers 2:1

21
Q

Symptoms in IPF

A

-Breathlessness 88%
-Dry cough 70% (bronchi stretches= irritable)

NOT
=Orthopnoea or PND
=Haemoptysis
=Wheeze
=Chest pains

22
Q

Clinical signs in IPF

A

-Bibasal fine mid and late inspiratory (velcro) crepitations in 90%
-Clubbing 60%

23
Q

Indication for CXR in primary care for IPF

A

-Aged 45 or older
-Persistent breathlessness
-With or without cough
-Bibasal chest crepitations

24
Q

Spirometry in IPF

A

-Restrictive disease= reduced FEV1 and FVC therefore normal or raised FEV1/FVC ratio
-Early disease= normal spirometry
-With co-existing emphysema, spirometry may be normal or even obstructive (FEV1/FVC reduced)

-Change in FVC is the current ‘gold standard’ biomarker for IPF disease progression: >10% decline in FVC over 6-12 months predicts poorer prognosis

25
Q

Lung volumes, gas transfer and walking tests in IPF

A

-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

26
Q

HRCT findings in IPF

A

-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

27
Q

When is lung biopsy performed?

A

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

28
Q

Risk of surgical lung biopsy in suspected IPF

A

-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

29
Q

Prognosis in IPF

A

-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

30
Q

2 drugs to slow rate of decline in IPF

A

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

31
Q

Mechanism of action of Nintedanib

A

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

32
Q

Side effects and tolerability of Pirfenidone

A

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

33
Q

Side effects and tolerability of Nintedanib

A

-Nausea
-Diarrhoea
-Dyspepsia
-20% discontinuation

34
Q

Non-pharmacological treatment of IPF

A

-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

35
Q

What is Sarcoidosis?

A

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

36
Q

Epidemiology of sarcoidosis

A

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

37
Q

Organ-specific symptoms of sarcoidosis

A

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

38
Q

Systemic general symptoms of sarcoidosis

A

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

39
Q

Pulmonary symptoms of sarcoidosis

A

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

40
Q

Examination findings of sarcoidosis

A

-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

41
Q

Staging in pulmonary sarcoidosis

A

-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
42
Q

Pulmonary function tests in sarcoidosis

A

-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

43
Q

Blood tests in sarcoidosis

A

-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

44
Q

Skin tests in sarcoidosis

A

-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

45
Q

Diagnostic biopsy in sarcoidosis

A

-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

46
Q

When to treat pulmonary sarcoidosis

A

-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)

47
Q

Treatment of pulmonary sarcoidosis

A

-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

48
Q

5 phases of steroid therapy in pulmonary sarcoidosis

A
  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

Respiratory (41 decks)

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