Interstitial Lung Disease + Sarcoidosis Flashcards

1
Q

What are Interstitial lung diseases?

A

Collective term for a group of disorders that cause inflammation and fibrosis disrupting the interstitium and the parenchyma of the lungs

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2
Q

How do patients with interstitial lung diseases typically present?

A

Chronic:
-SOB
-dry/non productive cough
-fatigue

Dry Inspiratory crackles
Finger clubbing
Chest pain
Weight loss?

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3
Q

What are the 2 main types of Interstitial Lung Disease?

A

Primary ILD
Secondary ILD

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4
Q

What is primary interstitial Lung disease?

A

Idiopathic/not caused by anything else

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5
Q

What is secondary interstitial lung disease?

A

When the interstitial lung disease is caused by something else

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6
Q

What is the most common cause of primary interstitial lung diseases?

A

Idiopathic pulmonary fibrosis / idiopathic interstitial pneumonia

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7
Q

What are some examples to ask a patient you think might have an interstitial lung disease?

A

SOB?
Cough non-productive?
Chest pain?
Occupational history
Family history and PMH
Acute on chronic? (Infectious signs)

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8
Q

What are some medications that can cause secondary interstitial lung disease?

A

Amiodarone
Bleomycin
Nitrofurantoin

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9
Q

What are some occupational causes of interstitial lung diseases?

A

Pneumoconiosis
Silicosis
Asbestosis
Byssinosis (cotton)

Hypersensitivity pneumonitis:
-bird fanciers lung
-cheese workers lung

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10
Q

What pre-existing medical conditions can cause pulmonary fibrosis?

A

Rheumatoid arthritis
Systemic sclerosis
Idiopathic myositis
Primary Sjögren’s syndrome
Mixed connective tissue disease
Sarcoidosis
Alpha-1-anti trypsin deficiency

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11
Q

What first line investigations would you do for a patient presenting with chronic SOB, chest pain and a dryer productive cough? (?ILD)

A

Routine bloods
ABG
O2 sats
6min walk tests
ECG
CXR

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12
Q

When you are then suspecting interstitial lung disease after doing your first line investigations, what further investigations will you do to help diagnose?

A

HRCT
Pulmonary function tests/Spirometryy
Echocardiography (pulmonary HTN)
Bronchoscope+- biopsy
Autoantibody screen (ANA, anti-Scl-70, rheumatoid factor, anti ro, anti la)
Bronchioalveolar lavage
Lung biopsy

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13
Q

What is expected to be seen on a HRCT of a patient with interstitial lung disease?

A

Bilateral bi-basal reticular shadowing
Honey combing
Ground glass opacity for idiopathic pulmonary fibrosis
Mediastinal lymphadenopathy

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14
Q

What type of result would you expect to see on pulmonary function tests/spirometry for an interstitial lung disease?

A

Restrictive curve (wizard hat) NOT SCALLOPING, that’s for obstructive

FEV1:FVC ratio above 0.7

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15
Q

How do you manage an interstitial lung disease?

A

Oxygen therapy
Cease smoking
Avoid cause
Treat autoimmune condition
Physiotherapist
Transplant if severe and young patient

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16
Q

What types of medications do you give to treat/manage idiopathic pulmonary fibrosis?

A

Antifibrotic agents

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17
Q

What are some anti-fibrotic medications you can give to slow the progression of idiopathic pulmonary fibrosis?

A

Perfenidone
Nintedanib

Both reduce fibrosis and inflammation.

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18
Q

What is the pathophysiology of hypersensitivity pneumonitis?

A

Extrinsic allergic alveolitis involving type III and type IV hypersensitivity reactions to inhaled allergens

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19
Q

How is a hypersensitivity pneumonitis diagnosed?

A

Bronchoalveolar lavage contains lymphocytes

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20
Q

How is hypersensitivity pneuomitis treated?

A

Oxygen therapy
Corticosteroids

21
Q

What is sarcoidosis?

A

Non caseating granulomatous mutlisystem disease that most commonly affects the lungs

22
Q

What are granulomas?

A

Inflammatory nodules containing macrophages

23
Q

What is the difference between the granulomatous inflammation seen in TB compared to sarcoidosis?

A

TB = caseating granulomatous inflammation
Sarcoidosis = non caseating granulomatous inflammation

24
Q

Who is sarcoidosis most common in?

A

Black women. 20-40 or 60+

25
Q

What parts of the body can sarcoidosis affect?

A

Lungs
Kidneys (stones)
Heart (Heart block)
MSK (arthritis)
GI
Cutaneous
CNS (facial palsy)
Ophthal (uveitis)

26
Q

What are some extra pulmonary skin changes that are often seen with sarcoidosis?

A

Erythema nodosum
Lupus pernio

27
Q

What is erythema nodosum and where is it commonly seen with sarcoidosis?

A

Nodules of inflammed subcutaneous fat on the shins

28
Q

What is lupus pernio seen in sarcoidosis?

A

Specific feature of sarcoidosis
Raised purple skin lesions on chin and nose

29
Q

What affect does sarcoidosis have on the lungs?

A

Mediastinal lymphadenopathy
Pulmonary nodules
Pulmonary fibrosis

30
Q

What systemic effects can sarcoidosis have on the body?

A

Fever
Malaise
Weight loss

31
Q

What affect can sarcoidosis have on the liver?

A

Liver noodles
Cholestasis
Cirrhosis

32
Q

What effect can. Sarcoidosis have on the eyes?

A

Uveitis
Conjunctivitis
Optic neuritis

33
Q

What affect can sarcoidosis have on the heart?

A

Bundle branch block
Heart block
Myocardium involvement

34
Q

What affect can sarcoidosis have on the kidneys?

A

Stones
Intersitial. Nephritis

35
Q

What affect can sarcoidosis have on the CNS?

A

Diabetes Insipidus (pituitary)
Nodules

36
Q

What affect can sarcoidosis have on the MSK system?

A

Arthritis
Arthralgia
Myopathy

37
Q

What is lofgrens syndrome?

A

Triadic presentation for sarcoidosis

-Erythema nodosum
-bilateral hilar lymphadenopathy
-poly Arthralgia

38
Q

What investigations would you do for a patient who you think might have sarcoidosis?

A

Routine bloods
LFTs (liver or gall bladder involvement?)
U+Es (kidney involvement, checking for Hypercalcaemia)
Urinalysis (Hypercalciuria, urine albumin-creatinine ratio for proteinuria)
ECG and ECHO if cardiac involvement?
Ophthalmology if eye involvement

SERUM ACE

39
Q

If you could only do 2 investigations for sarcoidosis, which ones would you do?

A

Serum ACE levels (is it elevated)?

Hypercalcaemia?? U+Es

40
Q

What imaging would you request to investigate a potential sarcoidosis?

A

Initially a CXR
Then a HRCT if needed

US if you think liver or kidney involvement

41
Q

Why is serum ACE a good investigation to determine the likelihood of sarcoidosis?

A

ACE expression increased with granulomas

42
Q

How many stages of classification are there for sarcoidosis based on a CXR?

A

4:
I
II
III
IV

43
Q

What is stage I of sarcoidosis on a CXR?

A

Bilateral hilar lymphadenopathy/lymph involvement without lung involvement

JUST LYMPH NODES

44
Q

What is stage II of sarcoidosis on a CXR?

A

Bilateral hilar lymphadenopathy + Pulmonary infiltrations/lung involvement

(Lymph nodes and parenchymal involvement)

45
Q

What is stage III of sarcoidosis on a CXR?

A

Pulmonary infiltrations without bilateral hilar lymphadenopathy (lymph nodes regress and its mainly lung parenchyma involved)

46
Q

What is stage IV of sarcoidosis on a CXR?

A

Pulmonary fibrosis

47
Q

What is the management of sarcoidosis?

A

Smoking cessation

Oral corticosteroids first line (6months-24months):
-Bisphosphonates + Calcium + Vit D
-PPIs

2nd line:
-methotrexate

48
Q

What may be considered if patietn has severe pulmonary disease with sarcoidosis?

A

Lung transplant