Interstitial lung disease Flashcards

1
Q

How is NSIP pattern different? Radiologically and clinical implications?

A

Still basal predominant but ground glass without evidence of honeycombing

Look CAREFULLY for underlying CTD

Often responds to steroids.

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2
Q

What is cryptogenic organising pneumonia? Radiologically what is specific about it?

A

Idiopathic form of bronchiolitis obliterans organisim pneumonia which could be caused by ID, CTD, drugs

See someone who look slike they had CAP but then the sx keep going for 6-8 weeks

SEE PERIPHERAL PREDOMINANCE IN HALF

Good prognosis with steroid response

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3
Q

What are the three interstitial smoking related lung diseases?

A

Respiratory bronchiolitis assoc ILD (centrilobular microlobular disease)
Desquamative interstitial pneumonia (Micronodular disease with ground glass all over)
Pulmonary langerhan cell histiocytosis (upper one cysts and nodules)

Subacute progressive cough and dyspnoea

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4
Q

Most common lung problem in RA?

A

Pleural involvement most common

Rheumatoid nodules
ILD- UIP worse prognosis- also see NSIP, BOOP
Isolated pulmonary hypertension
Methotrexate, leflunomide effects

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5
Q

pLAM is another diffuse lung disease- what do the effusions look like?

A

Often this disorder presents with a pneumothorax in premenopausal women or chylous effusion- ddx lymphoma

Due to an inactivating mutation of TSC gene causing activation of mTOR. Reccurs post transplant.

Can also have renal angiomyolipomas which rarely transform to sarcomas

Related to tuberous sclerosis

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6
Q

What are the seven idiopathic intersitial pneumonias?

A
NSIP
UIP
AIP
COP
LIP
DIP
RB assoc ILD
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7
Q

One sentence about NSIP

A

Do better than UIP, have relative subpleural sparing, NO HONEYCOMBING, no fibroblastic foci and temporally uniform on biopsy, more steroid responsive, more likely to be the one you see in scleroderma lung

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8
Q

UIP one sentence

A

The classic middle aged male smoker, histo shows temporally all different with fibroblastic foci, honeycombing, the dominant RA pattern

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9
Q

AIP one sentence

A

pathological equivalent of ARDS, 50% mortality, hyaline membranes on histo

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10
Q

COP one sentence

A

good prognosis, subpleural fibrosi with ground glass and nodules, often postinfective after IV abx but keep being sick. Can be assoc with CTD but then its BOOP as no longer cryptogenic

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11
Q

One sentence LIP

A

seen in women

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12
Q

DSIP

A

smoking asssociated, the end stage of RB-ILD, no honeycombing or fibrosis just lower lobe infiltrates, loads of macrophages in alveoli

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13
Q

RB-assoc ILD one sentence

A

Reticular lung changes, mosaic obstruction small airways

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14
Q

Whats the cut off for transplant referral?

A

DLCO 40% or below refer if appropriate

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15
Q

Three drugs that induce parenchymal lung disease?

A

Amiodarone- such a long half life that stopping drug rarely allows improvement
Methotrexate- good prognosis if stop drugs
Nitrofurantoin- CXR looks like failure in acute form

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16
Q

What time frame post ratiotherapy is radiation pneumonitis?

A

6 WEEKS
can be even outside the field ie other breast
steroids early on can help

17
Q

What are the two new antifibrotic drugs?

A

Nintendanib - inhibits TK 1,2
GI SE

Pirfenidone - photosensitive rash

Reduce rate of decline FVC
Reduce all cause mortality