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Respiratory (Long) > Interstitial lung disease > Flashcards

Interstitial lung disease Flashcards

1
Q

ILD - history?

A

P: dx date, initial symptoms, baseline ET

R: occupational exposures (dust [silica, asbestos, coal, berryllium], chemical fumes, bird, grain dust [farmer’s lung]), CTD (RA, scleroderma, AnkSpond, sarcoid), infection (TB, ABPA), RTx, TB, drugs (amiodarone, nitrofurantoin, MTX)

I: was it radiological or histological dx?

C: disease - Hospital admissions, ICU, infections, respiratory failures; drugs - from steroids, immunosuppressants

M: previous immunosuppressants, steroids, anti-fibrinolytic (Pirfenidone), TKI (Nintedanib), LTOT

C: current symptoms, latest PFT (TLC, DLCO), 6MWT, RVF symptoms & TTE - how is it impacting you?

Prognosis & insight

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2
Q

What are the 4 classifications of ILD?

A

ILD with known cause (occupational, drugs, CTD)

Granulomatous ILD (sarcoid, hypersensitivity, TB)

Idiopathic interstitial pneumonia (IPF, NSIP, RBILD - respiratory bronchiolitis ILD, which contains COP, AIP, DIP)

Others (Langerhan cell histiocytosis)

COP - Cryptogenic Organising Pnuemonia (BOOP - bronchiolitis obliterans organising pneumonia); AIP - acute interstitial pneumonia, DIP - desquamous interstitial pneumonia

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3
Q

Methotrexate & Nitrofurantoin associated ILD - when do they occur and tx?

A

MTX - can occur any time. Prognosis is good with withdrawal of the drug. Consider steroids

Nitrofurantoin - can occur any time, including even after previous uneventful use of the drugs. Cease drug and steroids can be helpful in chronic cases.

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4
Q

What is a typical feature of BOOP/COP (cryptogenic organising pneumonia, aka bronchiolitis obliterans with organising pneumonia). How do you treat it?

A

Occurs over weeks to months, presents with infection type symptoms with cough, fever, malaise and myalgia.

CT shows single or multifocal airspace opacifications

It has a better prognosis and responds to Steroids.

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5
Q

Which ILDs subtypes have strong association with smoking (representing possible smoking induced immune response)? (3)

A

RBILD (respiratory bronchiolitis ILD)

DIP (desquamative interstitial pneumonia)

Langerhans cell histiocytosis

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6
Q

Which CTDs. can cause ILD? (6)

A

RA

Ankylosing spondylitis

Sjogrens

Systemic sclerosis

Dermatomyositis/polymyositis

Polyarteritis nodosa (PAN)

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7
Q

What are the drug causes of ILD? (10, 4 categories)

A

Cardiac: procainamide, hydralazine, amiodarone

Rheum: D-penicillamine, MTX

Chemo: busulphan, bleomycin, cyclophosphamide

Others: nitrofurantoin, bromocriptine

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8
Q

ILD - things to comment on?

A

With regards to ILD

Comfortable on air +/- exertional SOB

Finger clubbing, cyanosis

Features of other CTDs / steroids side effects

fine, late inspiratory crackles - lower vs. upper lobe predominance

Pulmonary HTN

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9
Q

What is your approach to investigating this patient with ILD?

A

T: confirm the dx - HRCT to look for typical patterns c/w UIP (honeycombing in basal / peripheral predominance) or NSIP (GG opacificaiton with heterogeneous reticular changes), PFT (reduced TLC and DLCO - % change from baseline FVC (10%) or DLCO (15%) indicates significant decline). If Dx unclear, consider bronchostopy /BAL (or VATS) to rule out malignancy, infection, sarcoid…etc. Lavage looking for lymphocytosis (suggest drug induced or granulomatous), eosinophils

E: identify underlying aetiology - ANA, ENA, ANCA, CK, RhF, Scl-70, myositis panel)

S: ESR, ABG (TIRF), 6MWT (functional impaiment)

Tx baseline: FBC, EUC, LFTs

Screen complication: TTE (pHTN)

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10
Q

UIP pattern? (4)

A

Basal/subpleural predominance

Reticular changes

Honeycombing +/- traction bronchiectasis

Absent of changes inconsistent with UIP

Patient with diagnosed IPF. On HRCT images typical UIP pattern: Reticulation with honeycombing (○) and traction bronchiectasis (●) in basal and subpleural distribution (*).

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11
Q

What are pattern inconsistent with UIP pattern in ILD? (4)

A

Upper lobe / middle / peribronchovascular predominance

Extensive ground glass changes

Diffuse micronodules or cysts

Mosaic attenuation or air trapping

Patient with diagnosed IPF. On HRCT images typical UIP pattern: Reticulation with honeycombing (○) and traction bronchiectasis (●) in basal and subpleural distribution (*).

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12
Q

What are the causes of pulmonary infiltrate and eosiniphilia?

A

PLATE

Prolonged pulmonary eosinophilia (usually from drugs)

Loeffler’s syndrome

ABPA

Tropical (microfilaria)

Eosinophilic pneumonia & EGPA

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13
Q

What is your approach to managing this patient with interstitial lung disease?

A

Goal: Identify & treat acute exacerbation, maximise functional capacity, prevent complications

Confirm dx: HRCT, PFT, review biopsy if any. Review investigations for aetiology (ANA,ENA,RhF…etc). If active disease suspected, repeat HRCT + CTPA (PE), consider bronchoscopy/BAL to exclude infection.

A: treatment of underlying conditions, treat exacerbating factors - infection, GORD, OSA, depression

T: non-pharm

  • Educate: irreversible nature of disease, EOL and Advanced Care Directives early.
  • Remove exposure - drugs, stop smoking (Can stabiliese/improve lung function in RBILD)
  • Lifestyle - moderate exercise, healthy diet (involve dietician if malnourished), avoid alcohol
  • Infection prevention - food/hand hygine, avoiding contacts, 23-valent pneumococcal vaccine, flu vaccines
  • Pulmonary rehab - improves SOB and improved 6MWT
  • LTOT
  • Consider lung transplant

T: Pharm

  • IPF: Antifibrotic agent - pirfenidone, nintedanib (TKI) - both reduces decline in FVC (slows progression) - IMPULSIS trial
  • If evidence of acute exacerbation, broad spectrum ABx (based on previous cultures) + Prednisolone 1mg/kg
  • Consider steroids for NSIP, RBILD, BOOP/COP

Ensure F/U and screen complication (3-6 monthly)

Clinical exam, TTE for pulmonary HTN & cardiovascular disease, malignancy (5-fold increase), depression, drug side effects (steroids, pirfenidone, nintedanib), assess need for O2, involvement of palliative care early + advanced care directive.

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14
Q

Prescribing criteria for ILD treatments with pirfenidone and nintedanib?

  • hot topic as these drugs are on PBS.
A

Criteria for starting.

  1. Diagnosis of IPF
  2. FVC >50%
  3. FEV1/FVC >70%
  4. DLCO >30%
  5. No other causes found

If values are less than above, you have missed the boat and drugs won’t work.

Remember 30-50-70

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15
Q

Side effects of Pirfenidone & Nintedanib side effects to monitor in FU?

A

Pirfenidone - Photosensitivity

Nintedanib - diarrhoea

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16
Q

Do you think this patient’s idiopathic pulmonary fibrosis is active? How would you investigate & manage?

A

I suspect this patient may (or may not) have acute exacerbation / or active disease based on his/her clinical presentation.

I would like to investigate this further by performing HRCT looking for new bilateral ground glass opacification and/or consolidation superimposed on background of known fibrotic changes from previous HRCT.

Exclude PE, HF and infection (TTE, CTPA, septic work-up)

Bronchoscopy + BAL to evaluate for ling infection (if tolerable)

Mx: Methylprednosolone 1g/day or Prednisolone 1mg/kg + broad spectrum ABx with atypical cover. Early discussion of ceiling of care (intubation) given poor prognosis (3-4 months), Palliative consult.

17
Q

What is the overall prognosis of IPF for this patient?

How would you objectively assess this patient’s prognosis?

A

Median survival is between 2-5 years (~4 years on average), but 20-25% lives beyond 10 years.

Can use longitudinal clinical course - ≥10% decline in FVC (in 6-12 months), acute exacerbation, hospitalisations → poor short term survival. Can follow trajectory based on FVC/DLCO decline and regularly update the prognosis.

The most widely used validated clinical prediction model is GAP model (gender, age, physiology).

This is based on age, gender, FVC, DLCO and stages the disease to I-III to predict 1, 2, 3-year mortality.

18
Q

So what constitutes GAP index for IPF + and how do you score to stage the IPF?

A

Gender: 0 for female, 1 for male

Age: ≤60 (0), >65 (2)

Physiology

FVC (% predicted): >75% (0), 50-75% (1), <50% (2)

DLCO: >55% (0), 35-55% (1), ≤35% (2), cannot perform (3).

0-3 → stage I

4-5 → stage II

≥6 → stage III

19
Q

What is the 2-year mortality based on GAP stage?

A

Stage 1: 10%

Stage 2: 30%

Stage 3: 60%

20
Q

When would you consider lung transplantation in ILD? (5)

A

Evidence of UIP (can consider NSIP as outcome also improves) and

DLCO <40% decline or ≥10% decline in 6 months

FVC <80% or ≥15% decline in 6 months

Sat <88% on 6MWT

Pulmonary HTN

SOB or functional limitation attributable to ILD

Respiratory (Long) (11 decks)

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