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ICC1 > interstitial ling disease > Flashcards

interstitial ling disease Flashcards

1
Q

which area exactly is the interstitium of the lung ?

A

the area between the alveolar epithelium and pulmonary vascular epithelium

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2
Q

what are the different groups of interstitial lung diseases?

A

ILD of known association
granulomatous ILD
Idiopathic interstitial pneumonia
miscellaneous ILD

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3
Q

what are the types of exposure related ILD?

A

pneumoconiosis
drug-induced
connective tissue disease
radiation pneumonitis

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4
Q

what kind of drugs can induce ILD ?

A

amiodarone
methotrexate
nitrofurantoin

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5
Q

what are the differential diagnosis with ILD ?

A

infectious causes :
community acquired pneumonia + atypical pneumonia
chronic or remote mycobacterial infections
non-infectious possibilities:
lymphoproliferative disorders, cardiac pathology, acute respiratory distress syndrome

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6
Q

what are the symptoms associated with ILD ?

A 
dyspnea
cough 
fever ( more in an acute attack)
hemoptysis 
chest pain/pleurisy
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7
Q

what would a smoking history suggest in terms of ILD ?

A

Idiopathic pulmonary fibrosis
Respiratory bronchioloitis-associated interstitial lung disease (RBILD)
Desquamating interstitial pneumonitis (DIP)
pulmonary langerhans cell histocytosis (PLCH)

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8
Q

which ILD is less likely to be associated with smoking ?

A

Sarcoidosis and hypersensitivity pneumonitis

hypersensitivity pneumonitis

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9
Q

what ILD is associated with bird breeders and farmers ?

A

hypersensitivity pneumonitis

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10
Q

what ILD is associated with ceramic workers?

A

silicosis

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11
Q

what ILD is associated with pipe fitters and shipyard workers?

A

asbestosis

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12
Q

what ILD is associated with coal miners ?

A

coal workers’ pneumoconiosis

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13
Q

what pathology is amiodarone associated with ?

A

pulmonary fibrosis

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14
Q

what pathology is associated with nitrofurantoin?

A

alveolar haemorrhages

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15
Q

what symptoms are more suggestive of collagen vascular diseases ?

A

Raynaud’s phenomenon
proximal muscle weakness
joint swelling/pain

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16
Q

what does mild inspiratory squeak in physical examination suggest ?

A

suggests airway centred diseases:
constrictive bronchiolitis
hypersensitivity pneumonitis

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17
Q

what is the main finding on auscultation with ILD?

A

classic velcro rales or inspiratory crackles

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18
Q

what kind of Lab investigations are ordered for ILD ?

A 
CBC 
liver function
calcium, liver function test and a urine analysis 
along with immunological tests:
ssDNA and complement levels in SLE 
Anti-SCL 70 for diffuse scleroderma
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19
Q

what is the current gold standard for imaging in ILD ?

A

HRCT

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20
Q

what are the radiological findings in idiopathic pulmonary fibrosis ?

A

1- X-ray: reticular opacities
2- HRCT : traction bronchiectasis
bilateral, basal honeycombing

bilateral, basal honeycombing

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21
Q

what are the radiological findings in sarcoidosis on X-ray and HRCT ?

A

HRCT: septal bleeding
nodules next to bronchovascular bundles

X-ray: hilar lymphadenopathy

nodules

22
Q

which ILD show cystic changes with imaging ?

A

LAM

PLCH

23
Q

what is the hallmark of ILD ?

A

restrictive changes in pulmonary physiology

24
Q

what is the cause of the hypoxia inn patients with ILD?

A

ventilation perfusion mismatch

25
Q

what to do if gas exchange appears normal at rest ?

A

do exercise testing ( 6 minute walk test) to unmask the defects

26
Q

when can bronchoalveolar lavage be useful when it comes to ILD ?

A

useful in ruling out malignancies and infections

27
Q

bronchoscopic biopsies are of limited utility in the evaluation of ILD except with ….

A

sarcoidosis

28
Q

what is the treatment option for ILD?

A 
providing oxygen to hypoxemic patients 
pulmonary rehabilitation 
pneumococcal and influenza vaccination 
remove offending environmental agents 
corticosteroids and immunosuppressants
29
Q

what specific indications should be taken with LAM patients ?

A

avoid pregnancy , oophorectomy, progesterone, antiestrogens
give sirolimus

30
Q

what is more commonly seen after the age of 50 ?

A

idiopathic pulmonary fibrosis

31
Q

where is the affection in chronic hypersensitivity pneumonitis ?

A

upper lobe

32
Q

where is the affection in acute hypersensitivity pneumonitis ?

A

lower lobe

33
Q

when do we hear an expiratory wheeze refractory to inhaled bronchodilators ?

A

constrictive bronchiolitis

34
Q

what is the sign seen on HRCT associated with hypersensitivity pneumonitis ?

A

head cheese sign

35
Q

what is seen on HRCT of lymphangioleiomyomatosis LAM ?

A

thin walled cysts

36
Q

what is seen on HRCT off PLCH ?

A

diffuse variable sized
bizarre shaped cysts

37
Q

what is PAP ?

A

pulmonary alveolar proteinosis , accumulation of surfactants within the alveoli

38
Q

what is seen on HRCT in PAP ?

A

ground glass opacities demarcated by thickened interlobular septae
crazy paving ( variable alveolar filling )

39
Q

what is the appearance of BAL associated with pulmonary alveolar proteinosis ?

A

BAL of PAP appears as a milky fluid with debris and foamy macrophages

40
Q

what are the results of flow cytometric analysis of lymphocytes in sarcoidosis vs Hypersensitivity pneumonitis ?

A

hypersensitivity pneumonitis : CD4/CD8 < 1
sarcoidosis : CD4/CD8 > 2.5

41
Q

an increase in neutrophil count shows a worse prognosis in which diseases ?

A

sarcoidosis
hypersensitivity pneumonitis
idiopathic pulmonary fibrosis

42
Q

what method of obtaining a tissue sample is preferred when it comes to ILD ?

A

thoracoscopy guided and open lung biopsy

43
Q

when can we perform plasmapheresis ?

A

diffuse alveolar hemorrhage

44
Q

what is the treatment for PLCH ?

A

cladribine

45
Q

what is the treatment for PAP ?

A

whole lung lavage
GM-CSF therapy

46
Q

definitive management for any end stage lung ?

A

lung transplant

47
Q

what type of granulomatous disease is sarcoidosis ?

A

non caseating granuloma

48
Q

what is the most common presentation associated with sarcoidosis ?

A

hilar and left paratracheal adenopathy
positive despine sign

49
Q

what are the markers positive in pulmonary langerhans cell histocytosis

A

CD1a and S100

50
Q

what is the management in LAM ?

A

avoid pregnancy and give sirolimus

ICC1 (50 decks)

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