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Flashcards in Rheumatologic pleuropulmonary disease Deck (91):
1

What are the 5 rheumatologic diseases that most commonly have pleuropulmonary disease?

systemic sclerosis, systemic lupus erythematosus,
rheumatoid arthritis, dermatomyositis/polymyositis
and Sjogren syndrome

2

____ has more lung disease than any other autoimmune connective tissue disease.

Systemic sclerosis

3

What are the two forms of systemic sclerosis?

interstitial lung disease, vascular disease

4

About ____%of systemic sclerosis patients
get interstitial lung disease.

67

5

About ___% of systemic sclerosis patients
get pulmonary hypertension

20

6

What 2 things comprise the interstitial lung disease that people with systemic sclerosis get?

NSIP pattern and interstitial fibrosis

7

What are the systemic sclerosis mediators?

IL-8: [early] (neutrophil attractant and activator)
TNF: [early] (neutrophil attractant and promoter of neutrophil-endothelial binding)
MIF1alpha: (promoter of neutrophil chemotaxis)
RANTES: T cell recruiter and activator
Endothelin 1: vasoconstrictor
TGF-B: late, promoter of fibroplasia

8

What part of the lung is favored by scleroderma lung?

bases, posterior, periphery

9

What does CT show for scleroderma lung (early and late)

Early: ground glass, late: reticular infiltrates

10

____ is the most common pattern of early scleroderma lung disease.

NSIP pattern

11

Describe NSIP pattern.

lymphocytes and macrophage infiltrating interstitium followed by fibroblasts + collagen creating interstitial fibrosis. Lesions are homogenous.

12

Can scleroderma ever present with UIP pattern?

Yes!

13

What are some differences between UIP pattern and NSIP pattern?

NSIP will be homogenous wherease UIP is not. NSIP is associated more with ground glass and UIP is more honeycomb pattern, UIP doesnt seem to respond to therapy compared to NSIP pattern and has worse prognosis and may be due to aspiration.

14

What is seen histologically with systemic sclerosis vascular disease?

vascular disease: concentric
thickening and fibrosis of small pulmonary arteries

15

What is it called when vascular disease occurs without scleroderma lung?

associated with anti-centromere antibodies and limited systemic sclerosis, CREST syndrome

16

What are the 2 most common symptoms of systemic sclerosis lung disease? Why do they occur

Dyspnea, dry cough
- Dyspnea is due to increased work of breathing sensed by respiratory muscle mechanoreceptors

17

What are signs for systemic sclerosis lung disease?

Dry inspiratory velcro crackles especially at the bases initially subtle fine thought to represent the sudden opening of small airways abnormally closed by the pressure of interstitial inflammation, edema and fibrosis around them

18

How do you make diagnosis of systemic sclerosis?

Association of symptoms, signs and radiology
of interstitial lung disease with skin, esophageal and renal manifestations of scleroderma and/or
anti-Scl70 (anti-DNA topoisomerase) antibodies (40%). Also decreased DLCO and abnormal PFTs

19

How do you treat systemic sclerosis?

Cyclophosphamide. Not steroids- could bause scleroderma renal crisis

20

What is the prognosis for systemic sclerosis?

Mean survival is 12 years. Death rate higher for females.

21

What is the most common cause of death in systemic sclerosis?

Scleroderma lung disease 60%

22

What pleuropulmonary diseases are seen with lupus?

pleuritis, acute lupus pneumonitis, NSIP, pulmonary vascular disease (concentric arterial thickening, pulmonary thromboembolism), shrinking lung syndrome

23

What is the most common pleuropulmonary manifestation of lupus?

pleuritis- present in 20% at onset, at least 50% over time and up to 100% at autopsy

24

What symptoms are present with pleuritis in lupus?

often asymptomatic but sometimes with pleuritic chest pain

25

Describe the pathology of lupus pleuritis

typically fibrinous. may be associated with serosanguinous exudative pleural effustion. May have ANA in pleural fluid. There may be few inflammatory cells in the exudate. Pleural effusion usually small and bilateral.

26

T or F. Pleuropulmonary disease of lupus is broader spectrum and more commonly acute than any other autoimmune connective tissue disease.

T

27

How common is acute lupus pneumonitis in lupus?

rare

28

Describe the pathology of acute lupus pneumonitis.

Form of acute lung injury (DAD), can take form of diffuse hemorrhage, alveolitis with loose fibrin exudate and lymphocytes and macrophages in airspaces and a few lymphocytes in interstitium. Goes on to interstitial pneumonia and fibrosis.

29

What is later pathology of acute lupus pneumonitis?

goes on to interstitial pneumonia and fibrosis

30

Symptoms of acute lupus pneumonitis

dyspnea, fever, cough

31

Signs of acute lupus pneumonitis

fever, wet pulmonary crackles

32

CT of acute lupus pneumonitis

diffuse ground-glass opacification with pleural effusions 50% of the time

33

How do you treat acute lupus pneumonitis?

steroids and immunosuppresion

34

What is the prognosis of acute lupus pneumonitis?

50% mortality

35

How common is NSIP with lupus?

present 2% of lupus pt at onset, less than 5% overtime

36

How do you treat NSIP with lupus?

steroids

37

How common is pulmonary vascular disease with lupus?

uncommon, but 10% of lupus patients have subclinical evidence of pulmonary hypertension on echocardiography

38

What are the two types of pulmonary vascular disease with lupus?

concentric arterial thickening or thromboembolic disease

39

_____ associated with lupus anticoagulant or anti-phospholipid antibodies

Thromboembolic disease with lupus

40

What forms of lupus pleuropulmonary disease can cause hemoptysis?

1. pulmonary infarction due to PE
2. hemorrhagic acute lupus pneumonitis

41

Describe chronic pulmonary thromboembolic disease (with lupus)?

pulmonary artery webs, strands of fibrous tissue spanning the lumen, old pulmonary emboli that are organized. Recanalizing occurs.

42

Describe shrinking lung syndrome with lupus.

rare due to diaphragmatic weakness. Syndrome includes dyspnea, small lungs on x-ray, decreased DLCO, restrictive pattern PFT abnormalities. Usually self-limited

43

What types of pleuropulmonary disease are associated with RA?

1. pleuritis +/- pleural effusion
2. fibrosing interstitial lung disease
3. follicular bronchiolitis
4. obliterative bronchiolitis
5. organizing pneumonia
6. acute lung injury
7. pulmonary hemorrhage
8. rheumatoid nodules

44

Who is pleuropulmonary disease with RA more common in?

Smokers, 3x more common in smokers

45

How common is pleuropulmonary disease with RA?

14% have significant disease, 44% have subclinical disease

46

How common is pleuritis with fibrinous pleural effusion in RA?

20% of RA patients have pleuritic pain, less than 5% have an effusion, but 50% have pleural disease at autopsy

47

Describe interstitial pneumonia in pt with RA.

Has UIP or NSIP pattern in significant and likely similar proportions.

48

What are findings suggestive of RA-related interstitial disease?

abundant lymphocytes and germinal centers

49

You see a histo slide with germinal centers. What should you be thinking about?

RA-related interstitial disease

50

Pleuropulmonary disease in RA commonly involves what part of the lungs?

More commonly pleura but also bronchioles

51

How do RA pt with interstitial lung disease present on exam?

exertional dyspnea, inspiratory velco crackles, decreased DLCO, interstitial fibrosis on CT

52

Describe the UIP pattern of fibrosing interstitial lung disease.

Temporally heterogenous with early areas of only inflammation, late areas of only fibrosis, and everything inbetween. Most severe in periphery, lower lobes. Fibrotic lung remodeling --> honeycomb lung

53

What is the end stage of all fibrosing interstitial lung disease?

Honeycomb

54

Histologically, what can you expect to see with later stage interstitial pneumonia in RA?

Mucinous microcysts (honeycomb change) and lymphoid follicles (germinal centers)

55

Describe follicular bronchiolitis.

It is more common with RA. CT shows peri-bronchial or centrilobular nodules and ground glass opacities with broncho-centric distribution.

56

How do you treat follicular bronchilits in RA?

steroids

57

describe obliterative bonchilitis

More common in RA, obstructive patter PFTs, sometimes superimposed Sjogren's, does NOT respond to steroids.

58

Describe organizing pneumonia.

More common in RA, present with acute onset, fever, and alveolar infiltrates, responds to Steroids

59

Histological appearance of rheumatic nodules

rim of palisading histiocytes, basiphilic debris and surrounding fibrosis, lymphocytes, macrophages, and multinucleated giant cells

60

How common is pulmonary fibrosis in the death of pt with RA?

7/8 who died of resp failure had fibrosis!

61

Pneumontitis is a recognized adverse effect of methotrexate due to :

cytotoxicity causing diffuse alveolar damage or hypersensitivity causing chronic interstitial pneumonia with microscopic features similar to
usual interstitial pneumonia.

62

What does the histology of methotrexate pulmonary tox look like early on vs later?

early it is too bad with patchy lymphocytic interstitial infiltration. Late is bad and fibrotic

63

How severe is methotrexate pulmonary toxicity?

Life-threatening: up to 20% mortality

64

How should you treat methotrexate pulmonary toxicity?

steroid therapy

65

How does methotrexate pulmonary toxicity present?

most often subacute, in first 4 months. Presents with dyspnea, cough, fever, have bilateral pulmonary crackles

66

How does methotrexate pulmonary toxicity appear on CT?

can be bilateral, partly ground glass, partly reticular or can be coarser, denser, more reticular and worst posterior.

67

What are pulmonary toxicities for RA therapies besides MTX?

Gold: acute lung injury or chronic interstitial pneumonia.
Penicillamine: alveolar hemorrhage
Anti-tumor necrosis factor agents can cause interstitial pneumonia

68

Ways that polymyositis/dermatomyositis-associated lung disease is like other connective tissue diseases?

Usually chronic, Usually NSIP, Usually have dyspnea, Usually have ground-glass or reticular infiltrates, Usually have restrictive pattern PFT abnormalities and decreased DLCO, Can present with acute lung injury like lupus or rheumatoid lung disease, Treatment = steroids +/- immunosuppression

69

How is polymyositis/dermatomyositis-associated lung disease treated?

steroids and immunosuppresion

70

How common is polymyositis/dermatomyositis-associated lung disease?

Occurs in 70% of pt and is most common cause of death (like scleroderma)

71

How common is ILD and organizing pneumonia in polymyositis/dermatomyositis-associated lung disease?

50% of the 70% of pt who have lung disease

72

How does polymyositis/dermatomyositis-associated lung disease appear on CT in 50% of cases?

consolidation

73

T or F. Respiratory failure from muscle weakness is COD in 25% of pt with polymyositis/dermatomyositis-associated lung disease.

T

74

Muscle weakness can lead to what complications in polymyositis/dermatomyositis-associated lung disease?

Death, aspiration pneumonia

75

What is anti-synthetase syndrome and how common is it?

myositis+ arthritis + lung disease (20%)

76

T or F myositis is associated with MALIGNANCY.

T

77

How is Sjogren syndrome-associated lung disease
like other connective tissue diseases?

1. Most common symptom is dyspnea
(10% of Sjogren’s patients have it)

2. Most common CT scan finding is lower lobe
ground-glass (or reticular) opacities

3. Most common histopathology is
nonspecific interstitial pneumonia (NSIP)

4. Not much else
(a lot more differences than similarities)

78

How common is Sjogren syndrome-associated lung disease?

11% of pt

79

What part of the lung is involved in Sjogren syndrome-associated lung disease?

airways, especially submucosal glands

80

T of F xerotrachea and xerobronchea are associated with Sjogren syndrome-associated lung disease.

T

81

How does someone with Sjogren syndrome-associated lung disease get a dry relentless cough?

loss of submucosal gland secretion

82

When does Sjogren syndrome-associated lung disease resemble COPD?

bronchial hyper-responsiveness

83

What does histological Sjogren syndrome-associated lung disease look like?

it has germinal centers and is bronchiolocentric, has more lymphocytes, has macrophages and multinucleated cells

84

What is the relationship of lymphoma and Sjogren syndrome-associated lung disease?

The proliferating lymphocytes in Sjogren syndrome-associated lung disease can become semi or fully autonomous causing pseudolymphoma or lymphoma and can commonly be called lymphoproliferative disorders. 44x more common in these pt.

85

______ are all suggestive of lymphoma in Sjogren syndrome-associated lung disease.

CT: Mediastinal lymphadenopathy, pleural effusion, prominent nodularity of the lung lesions and lung cysts
Histo: expansile or tumefactive pattern of lymphocytic infiltration, tracking in a lymphangitic pattern, absence of follicles, macrophages and multinucleated giant cells

86

T or F Rheumatologic disease is commonly acute.

FALSE- it is not. Infection could be acute

87

T or F Rheumatologic disease is commonly nodular.

FALSE- it is not! Think fungal infection

88

What are intersitial infections that cause chronic pneumonias that could be confused with rheumatologic lung disease?

pneumocystic, sarcoidosis, t. gondii

89

What are nodular chronic pneumonias that could be confused with rheumatologic lung disease?

TB, histo, aspergillosis, cryptococcosis, coccidio, blasto

90

T or F Subacute or chronic dyspnea in a rheum pt is more likley rheum than infection.

T

91

6 characteristics commonly seen in autoimmune interstitial pneumonia

1. Chronic
2. Dyspnea (+/- cough)
3. Velcro crackles
4. Basal, posterior + peripheral
ground-glass or reticular infiltrates
5. Restrictive pattern PFTs
6. Decreased DLCO