ILD Flashcards

1
Q

Do people with ILD have an obstructed or restricted flow?

A

restricted

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2
Q

What are the three broad types of restricitve lung disease?

A

-extrapulmonary
-neuromuscular
-lung parenchyma

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3
Q

Name some causes of extrapulmonary lung conditions

A

-obesity
-scoliosis
-ascites

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4
Q

What is acities?

A

extra fluid in the abdomen

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5
Q

Name some causes of neuromuscular lung conditions

A

-myasthenia gravis
-motot neurone disease
-diaphragmatic paralysis

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6
Q

Name some causes of lung parenchyma lung conditions

A

-ILD

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7
Q

Name some ILD’s ( non idiopathic)

A

-drug induced lung disease
-sarcoidosis
-connective tissue disorders
-occupational lung diseases
-hypersensitivity pneumonitis
-most common type is IPF

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8
Q

What happens to the lungs with ILD’s?

A

-fibrosis (scarring) of the pulmonary interstitium and the bronchovascular and septal tissue

-may be inflammation

-alveolar airspaces and distal airways may be affected

-cause is often idiopathic

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9
Q

What are the four main catergories of ILD?

A

1-idiopathic interstitial pneumonitis ( IIP)
2-ILD due to specific causes
3-granulomatous ILD
4-rare causes

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10
Q

Describe the pathologic findings in IPF

A

-patchy, dense fibrosis +/- honeycombing
-very little inflammation
-areas of interspersed normal lung
-mixed picture of normal lung and affected areas

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11
Q

Describe the radiographic findings in IPF

A

-peripheral, lace-like areas of fine reticular opacity
-concentrated posteriorly in the bases of the lungs
-ground glass opacification (cloudy) of the lower lobe
-honeycoming and/or traction bronchiectasis

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11
Q

What is the treatment for IPF?

A

-no response to steroids or othr immunosuppressive drugs
-smoking cessation does not change course of disease
-course of disease is marked by periods of rapid clinical deterioration

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12
Q

What is the prognosis for IPF?

A

-5 year survival rate 10-50%
-median survival after diagnosis 2.5-3.5 years
-higher prevalence and mortality rates in men, who typically present age 50-70

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13
Q

What is the diagnosis for IPF?

A

-if clinical and radiographic findings are consistent and suggest IPF, a diagnosis can be made without a biopsy

-pathologic evidence is only necessary in cases where the diagnosis is uncertain

-all other forms of IIP require pathologic confirmation for diagnosis

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14
Q

Whats the difference between IPF v Non-usual interstitial pneumonitis ( Non-UIP)

A

-IPF has poor prognosis
-little effective therapy for IPF
-patients with Non- Idiopathic interstitial pneumonitis may respond to therapy
-smoking cessation may help may help Non- UIP nut NOT IPF

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15
Q

What is a video- assisted thoracoscopy ( VATS) ?

A

-keyhole surgery used to obtain lung biopsy samples

-performed under General anaesthetic

-small inscisions in the chest made close to the ribs. A thoracoscope os omseted to view the lungs

16
Q

Why are VATS used?

A

-taking biopsies from the lung or the lining of the chest, removing lymph nodes

-removing lumps of growths from the lungs

-to treat various lung condtions such as pleural effusion

17
Q

What drugs are known to cause lung damage?

A

-cancer chemotherapy ( bleomycin)
-drugs for heart conditions ( amiodarone)
-anti-inflam drugs ( methotrexates )
-antibiotice ( nitrofurantoin)
-paraquat
-therapeutic radiation
-recreational drugs

18
Q

What is amiodarone used for?

A

treating cardiac arrythmias

19
Q

Describe drug induced diffuse parenchymal lung disease

A

-patients should have RFTs prior to treatment with drugs known to damage the lungs

-if lung damage is suspected then the drug should be stopped asap

-steroids may help reverse the damage, but the damage may be irreversible

20
Q

What causes hypersensitivity pneumonitis?

A

-also knwon as extrinsic allergic alveolitis

-caused by immune mediated inflammatory response to inhaled antigens such as mould, dust or chemicals to which the patient becomes sensitized

21
Q

Name some diseases that are a result of hypersensitivity pneumonitis

A

-bird fanciers lung- bird droppings or antigens on bird feathers
-farmers lung - mould in hay

22
Q

What is connective tissue disease/ collagen vascular diseases?

A

-range of autoimmune conditions attacking collagen in connective tissue

diseases associated with ILD:
-rheumatoid arthiritis, sjogrens syndrome, scleroderma

23
Q

What is rheumatoid arthritis?

A

-autoimmune condition affecting joints causing inflam and pain

-inflam process in RA can involve other sites such as the lungs, heart, blood vessels and eyes

-lungs are common site after joints

-RA treatment may cuase drug-induced ILD

24
What is Sjogren's syndrome?
-chronic autoimmune condition -destroys glands that produce saliva and tears -generalised dryness
25
What is scleroderma/ systemic sclerosis?
-autoimmune condition causing skin hardening -in severe conditions can affect internal organs such as the lungs -causes ILD and also affects blood vessels leading to pulmonary hypertenstion
26
What is occupational lung disease?
-inhalation of mineral dusts such as asbestos, silica or coal, causing direct cytotoxic effect on lung parenchyma
27
Describe granulomatous ILD
-granuloma is a clump of tissue macrophages ( histocytes) -granulomas form as an inflam response to an immune reaction -sarcoidosis is the most common type
28
Describe sarcoidosis
-multisystem inflam disorder of unknown cause -deposition of granulomas in the lungs -Hilar lymphadenopathy- lymph nodes in the centre of the chest become inflammed -mild form-few systems -may cause restricive and/or obstructive defect
29
What are some rare causes of ILD?
-infiltrative diseases- amyloidosis -post inflam disease- ARDS -post infective disease- HIV -post bone marrow transplant -rare lung diseases- Langerhan's cell histiocytosis
30
List some typical presentations of IPF
-breathlessness -persistant/ non-productive cough -bilateral inspiratory crackles -decreased excersise tolerance with desaturation on excersise -fatigue -weight loss -fever -joint pain -clubbing
31
How do we diagnose ILD?
-full patient history and physical examination -respiratory function test -x-ray or CT scan -VATS biopsy -blood tests such as - rheumatoid factor,check for immune disease such as antibodies to avian precipitans, check for infection
32
Whats the effect of ILD on lung function?
-lungs have decreased compliance due to inflam infiltrates or increased fibrous tissue -thickening of alveoli makes it more difficult for gas exchange to occur -patient's lung volume is reduced as unable to expand the lungs -increased work to breathing -patients will take smaller and more rapid breaths than healthy subjects
33
What are some treatment options of ILD?
-pulmomary rehabilitation -long term oxygen therapy -lung transplant- 50% survival at 5 years -pallative care -drug treatment
34
Name some drugs used to treat ILD
-antitussive medication -pain relief -steroids -N-acetylcysteine -antifibrotic agents -immunosuppressive agents- azothiprine and methotrexate
35
What is N-acetylcysteine?
-a precursor to glutathione which is an antioxidant -mucolytic -thought to restore oxidant-antioxidant balance -given in combination with steroids and azathioprine -antifibrotic properties
36
When are immunosuppresive agents considered?
-patients who do not respond to steroids or have contraindications to high-dose corticosteroid therapy -Azathioprine is less toxic than methotrexate -can cause ILD
37
Describe antifibrotic agents
-pirfenidone and nintedanib -suitable for patients with mold to moderat disease, FVC berween 50 and 80% predicted, now also patients with FVC> 80% side effects- tiredness,weight loss, sensitivity to sunlight, dizziness, nauseas and vomiting -specialist centres