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Clin Med - Pulmonary > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

What is another name for interstitial lung disease (ILD)?

A

diffuse parenchymal lung disease (DPLD)

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2
Q

What is the typical chief complaint in an ILD patient?

A

dyspnea

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3
Q

What are the hallmarks of ILD?

A

disruption of the distal lung parenchyma characterized by infiltration of cellular and non-cellular material into the lung

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4
Q

What is DLPD of known cause?

A

occupational and environmental

drugs

posions

radiation

infectious

disorders of other organ systems (pulmonary edema and chronic uremia)

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5
Q

What is the hallmark of DPLD on lung testing?

A

reduced lung compliance

and reduced FRC (restrictive defect)

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6
Q

How is DLCO affected in DPLD?

A

It is reduced - fibrosis thickens the pulmonary membrane

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7
Q

what are ground glass opacities on chest xray?

A

water outside the vasculature (leaky lung vessels)

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8
Q

what are the signs of DPLD on chest xray?

A

diffuse parenchymal infiltrates (normal chest film in 10%)

patterns of fine nodules (granulomas)

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9
Q

What do PFTs show with DPLD?

A

restrictive pattern - low FRC/RV/TLC and low DLCO

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10
Q

What sign is present in >50% of IPF patients?

A

digital clubbing

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11
Q

what might you hear on auscultation in DLPD?

A

crackles at the bases

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12
Q

What medications are known to be common offenders in DLPD?

A
  • chemotherapy/radiation
  • antibiotics - furantonin, sulfasalazine
  • anti-arrhythmics: amiodorone, propanolol, tocainide
  • anti-inflammatory: gold, penicillamine
  • anti-convulstants: dilantin
  • Oxygen (?)
  • Illicit IV drugs (cut with talc)
  • crack cocaine inhalation
  • drug-induced SLE: procainamide, hyrdralazine
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13
Q

lymphadenopathy in the periphery suggests what DPLD disorders?

A

silicosis or sarcoidosis

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14
Q

If you get an abnormal chest xray, what test should you order next?

A

high resolution CT

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15
Q

What is the epidemiology of sarcoidosis?

A

commonly in African Americans (or Japanese)

F>M

Younger (20-50)

familial clustering

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16
Q

what are the common signs of sarcoidosis?

A

bilateral hilar lymphadenopathy

pulmonary infiltration

ocular and skin lesions

non-caseating epitheliod granulomas

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17
Q

How is the definitive diagnosis of sarcoidosis made?

A

histology and radiology show granulomas and other causes of granulomas (mycobacteria or fungi) have been ruled out

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18
Q

What disease causes sarcoid to go away?

19
Q

What is the prognosis for sarcoidosis?

A

depends on mode of onset and extent of disease

AA has worse prognosis

self-limited course in Lofgren’s syndrome - white males with E.nodusum and swollen shins

these guys do better without treatment

20
Q

what is a key difference in symptoms between TB and sarcoidosis?

A

no night sweats in sarcoidosis

21
Q

What are some of the constitutional symptoms in sarcoidosis?

A

fever - low grade

fatigue

malaise

weight loss

(1/3 patients)

22
Q

where does sarcoid tend to appear in the lungs?

A

upper lobes > lower lobes

23
Q

What is the mainstay of therapy for sarcoidosis?

A

systemic glucocortiocosteroids

24
Q

What is hypersensitivity pneumonitis?

A

immunologically -mediated reaction to inhaled small molecular weight antigens, e.g. fungi

(sometimes called Farmer’s lung; bird fancier’s lung; hot tub lung)

25
What are the signs and symptoms of hypersensitivity pneumonitis?
fever, malaise, flu-like symptoms cough dyspnea 4-8 hours after inhaling antigens - symptoms last 12-48 hours
26
what can happen with chronic exposure in hypersensitivity pneumonitis?
permanent fibrotic scarring of the lung tissue can occur
27
What is idiopathic pulmonary fibrosis?
a distinct DPLD that is a chronic fibrosing interstitial pneumonia of unknown cause
28
How is IPF diagnosed definitively?
By evidence of a histologic pattern of usual interstitial pneumonia (UIP) on lung biopsy
29
What is the epidemiology of IPF?
13-20/100k in US prevalence (rare) age of onset 50-70 (very rare before 40)
30
What's the prognosis for IPF?
Terrible - no real treatment 5 year mortality of 68%
31
What is the clinical presentation with IPF?
progressive dyspnea on exertion paroxysmal cough (usually non-productive) mid-to-late inspiratory crackles (ALWAYS at the bases) digital clubbing in 40-75%
32
What do PFTs look like in IFP?
restrictive pattern reduced DLCO widened P(A-a)D02 desaturation on exercise
33
What do you often see in IPF on CT?
honeycombing - areas where fibrotic tissue has remodeled normal lung tissue
34
What is Nonspecific Interstitial Pneumonitis (NSIP)?
Clinical appearance similar to IPF, but histologically distinct features no fibroblastic foci and more ground glass opacities on CT/Xray
35
What is the prognosis for NSIP?
Better than IPF - better response to steroids
36
what is the epidemiology of NSIP?
onset at midlife (40-50) no sex predominance usually gradual no association with cigarette smoking
37
What are the signs and symptoms of NSIP?
dyspnea cough fatigue 50% have weight loss basilar crackles clubbing less common than IPF (10-35%)
38
What is Respiratory Bronchiolitis-Associated Lung Disease?
intraluminal macrophages in 1st and 2nd order respiratory bronchioles in current smokers
39
What are the Sx of RB-ILD?
Usually mild dyspnea cough PFTs show restriction, DLCO reduced
40
What is the treatment for RB-ILD?
quit smoking - most people improve
41
What is Desquamative Interstitial Pneumonitis (DIP)?
A form of ILD seen in current or former cigarette smokers or environmental smoke exposure
42
What is the epidemiology of DIP?
mean age at presentation is 42 male\>female cigarette smoking history or exposure
43
What are the signs and symptoms of DIP?
insidious onset of dyspnea and dry cough
44
What is the prognosis for DIP?
27% mortality at 12 years smoking cessation helps most and systemic steroids complete remission possible

Clin Med - Pulmonary (13 decks)

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