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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?

A

HIV

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
Q

What are the signs and symptoms of hypersensitivity pneumonitis?

A

fever, malaise, flu-like symptoms

cough

dyspnea

4-8 hours after inhaling antigens - symptoms last 12-48 hours

26
Q

what can happen with chronic exposure in hypersensitivity pneumonitis?

A

permanent fibrotic scarring of the lung tissue can occur

27
Q

What is idiopathic pulmonary fibrosis?

A

a distinct DPLD that is a chronic fibrosing interstitial pneumonia of unknown cause

28
Q

How is IPF diagnosed definitively?

A

By evidence of a histologic pattern of usual interstitial pneumonia (UIP) on lung biopsy

29
Q

What is the epidemiology of IPF?

A

13-20/100k in US prevalence (rare)

age of onset 50-70 (very rare before 40)

30
Q

What’s the prognosis for IPF?

A

Terrible - no real treatment

5 year mortality of 68%

31
Q

What is the clinical presentation with IPF?

A

progressive dyspnea on exertion

paroxysmal cough (usually non-productive)

mid-to-late inspiratory crackles (ALWAYS at the bases)

digital clubbing in 40-75%

32
Q

What do PFTs look like in IFP?

A

restrictive pattern

reduced DLCO

widened P(A-a)D02

desaturation on exercise

33
Q

What do you often see in IPF on CT?

A

honeycombing - areas where fibrotic tissue has remodeled normal lung tissue

34
Q

What is Nonspecific Interstitial Pneumonitis (NSIP)?

A

Clinical appearance similar to IPF, but histologically distinct features

no fibroblastic foci and more ground glass opacities on CT/Xray

35
Q

What is the prognosis for NSIP?

A

Better than IPF - better response to steroids

36
Q

what is the epidemiology of NSIP?

A

onset at midlife (40-50)

no sex predominance

usually gradual

no association with cigarette smoking

37
Q

What are the signs and symptoms of NSIP?

A

dyspnea

cough

fatigue

50% have weight loss

basilar crackles

clubbing less common than IPF (10-35%)

38
Q

What is Respiratory Bronchiolitis-Associated Lung Disease?

A

intraluminal macrophages in 1st and 2nd order respiratory bronchioles in current smokers

39
Q

What are the Sx of RB-ILD?

A

Usually mild

dyspnea

cough

PFTs show restriction, DLCO reduced

40
Q

What is the treatment for RB-ILD?

A

quit smoking - most people improve

41
Q

What is Desquamative Interstitial Pneumonitis (DIP)?

A

A form of ILD seen in current or former cigarette smokers or environmental smoke exposure

42
Q

What is the epidemiology of DIP?

A

mean age at presentation is 42

male>female

cigarette smoking history or exposure

43
Q

What are the signs and symptoms of DIP?

A

insidious onset of dyspnea and dry cough

44
Q

What is the prognosis for DIP?

A

27% mortality at 12 years

smoking cessation helps most and systemic steroids

complete remission possible

Clin Med - Pulmonary (13 decks)

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