Diffuse Parenchymal Lung Disease Flashcards

1
Q

Interstitial lung disease is often times, called __________.

A

Diffuse parenchymal lung disease (DPLD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Diffuse parenchymal lung disease (DPLD) all have involvement of _________ on histopathology.

A

Distal lung parenchyma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How can we classify Diffuse Parenchymal Lung Diseases on histopathology?

A
  • 1. Inflammation and fibrosis
  • 2. Granulomatous changes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What histopathologic patterns have interstitial involvement?

A
    1. UIP (usual interstitial pneumonitis)
    1. NSIP (Nonspecific interstitial pneumonitis)
    1. BOOP (bronchiolitis obliterans organizing pneumonia)/ COP (cryptogenic organizing pneumonia)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What histological pattern is seen with UIP?

A

Heterogenous involement of lung with different stages of progression of fibrosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Usual interstitial pneumonitis is associated with several diseases or exposures including what?

A
  1. pneumoconioses
  2. radiation injury
  3. end-stage hypersensitivity pneumonitis
  4. advanced sarcoid
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

If no underlying process is ID’d, UIP is instead diagnosed as what?

A

Idiopathic pulmonary fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q
  • What pattern is seen in non-specific intersitial pneumonitis (NSIP) on histo?
  • CT?
  • Assx with what diseases?
A
  • Uniform involement of lung parenchyma, with cellular infiltartion or fibrosis.
  • Ground glass infiltrates
  • Autoimmune CT disorders
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What pattern is seen in BOOP/COP on histo?

CT?

Assx with what diseases?

A
  • Small airway bronchiolitis with granulomas + organizing pneumonia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

_________ granulomas are typical for sarcoidosis.

________ granulomas are typical for hypersensitivity pneumonitis.

A
  • well-formed non-caseating
  • loosely formed granulomas
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How are DLPD’s diagnosed?

A

Thorough hx, PE, lab and imaging studies, open lung biopsy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What symptoms raise the possibility of DPLD?

A
  1. Progressive dyspnea, over months
  2. Reduced excercise tolerance
  3. Persistant dry cough
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q
  • Idiopathic pulmonary fibrosis most often occurs in patients ______.
  • Interstitial lung disease assx with … occurs in patients ________
    • CT disease
    • Sarcoidosis
    • Lymphangioleimyomatosis
    • LCH
A
  • Over 50 YO.
  • 20-40 YO
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What DPLDs occurs most in females?

A

Lymphangioleimyomatosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What DPLDs are most associated with smoking?

A
  • 1. RB-ILD
  • 2. Desquamative intersitial pneumonia
  • 3. Langerhan cell histiocytosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

If patient has exposure history to birds, hay, mold or other organic material, what DPLDs should we consider?

A

Hypersensitivity pneumonitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

If patient has occupational exposure, what DPLDs should we consider?

A

1. Asbestosis

2. Silosis

18
Q

It patient has acute onset, what DPLDs should we consider?

A
  • 1. Acute Interstitial Pneumonia
  • 2. Acute eosinophilic pneumonia
  • 3. COP
  • 4. Hypersensitivity pneumonitis
  • 5. Drug-induced interstiial lung disease
  • 6. Diffuse alveolar hemorrhage syndrome
19
Q

What findings are indicative of sarcoidosis or CT diseases?

A
    1. Erythema nodosum
    1. Uveitis/conjunctivitis
    1. Arthritis
20
Q

What findings are indicative of sarcoidosis and Sjrogens syndrome?

A

Enlargement of lacrimal/salivary gland

21
Q

Lymphadenonapthy/hepatosplenomegaly is indicative of what DPLD?

A

Sarcoidosis

22
Q

Muscle weakness is associated with what DPLD?

A
  • Polymtositis
  • Dermatomyositis
23
Q

Clubbing is indicative of what DPLD?

A

Idiopathic pulmonary fibrosis.

24
Q

Hx of someone with suspected DPLD should be focused on what?

A

Looking for underlying causes

25
Q

What should docs look for on PE of DPLDs?

A
    1. Oxygenation status (desaturation with exertion, which is an early sign)
    1. Crackles on inspiration
    1. Clubbing
    1. Autoimmune diseases
    1. Right sided heart failure, a consequence of DPLD.
26
Q

What is used as a diagnostic evaluation of DPLD to tell us about distribution and extent of disease.

A

HRCT (High-resolution CT)

27
Q

What can lead to a diagnosis without the need for biopsy?

A

HRCT

28
Q

What will people with DPLD show on PFT?

A
  • Decreased lung volumes (total lung capacity, residual volume, functional residual capacity)
  • Decreased diffusing capacity
29
Q

Is a lung biopsy always needed to diagnosis DPLD?

If so, how?

A

No, not always.

Best performed via an open procedure using a thorascopic approach.

30
Q

What 2 diseases produce a granuloma response?

-All others will produce inflammation and fibrosis-

A

Sarcoidosis and hypersensitivity pneumonitis

    1. Drug-induced parenchymal lung disease
    1. Smoking-related DPLD
    1. Connective Tissue-Associated DPLD
    1. Pneumoconiosis
    1. Idiopathic Pulmonary Fibrosis
    1. Acute
31
Q

What drugs cause drug-induced parenchymal lung disease?

A
  • 1. Amiodorone
  • 2. Methotrexate
  • 3. Nitrofurantoin
32
Q

DPLD is associated with which CT disorders?

A
  • 1. RA
  • 2. Progressive systemic sclerosis (scleroderma)
  • 3. Polymyositis/dermatomyositis
  • 4. Sjogen syndrome
  • 5. Behcet disease
33
Q

When DPLDs is associated with asbestos, silicon or beryllium, they are called ______.

A

Pneumoconiosis

34
Q

Imaging of pneumoconiosis will show what?

A
  • Basilar and subpleural bilateral, linear intersitial markings
  • Calcified pleural plaques
35
Q

Idiopathic pulmonary fibrosis is important to diagnose, why?

A

Poor prognosis

36
Q

Histopathology of Idiopathic Pulmonary fibrosis shows what?

A

UIP

37
Q

Acute intersitial pneumonia is also called __________.

Imaging:

Diagnosis:

Histopathology:

A
  • Hamman-Rich syndrome
  • Bilateral alveolar disease with ground-glass change
  • Open lung biopsy
  • DAD (diffuse alveolar damage)
38
Q

When is organizing pneumonia termed BOOP or COP?

Tx?

A
  • Proximate cause is IDd => BOOP
  • No cause found => COP
  • Systemic glucocorticoids
39
Q

Hypersensitivity Pneumonitis

  • Occurs when?
  • HRCT shows
  • Histo shows:
  • Tx:
A
  • Repeated immunologic reaction to inhalation of antigens
  • Ground glass opacities with centrilobular nodues
  • Noncaseating granulomas
  • Avoid causes and steroids for chronic sx.
40
Q

Sarcoidosis

  • Is?
  • Age?
  • PFT shows
  • Imaging:
  • Histo shows:
  • Tx:
A
  • Formation of granulomas all over body, including lung that affects mostly blacks
  • Bimodal: Peaks over 18 YO and ppl 50-60
  • Restriction, reduced diffusing capacity, obstruction of airways
  • Bilateral hilar adenopathy and/or interstitial infilteates or patchy alveolar infiltrates
  • Bronchocentric noncaseating granulomas
  • Systmic glucocorticoids
41
Q

Many patients with severe/chronic DPLD develop what?

A

Pulmonary HTN

42
Q

Treatments for people with DPLD?

A
    1. Stop smoking
    1. Supp O2
    1. Symptomatic tx for breathing and treatment of infections
    1. Vasodilating agents to reduce right side vascular resistance