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Musculoskeletal > Pleuropulmonary Disease > Flashcards

Pleuropulmonary Disease Flashcards

1
Q

Systemic Sclerosis “scleroderma”

A
  • more lung disease than any other autoimmune connective tissue disease (collagen vascular disease)
    1) Interstitial lung disease
    2) Vascular disease
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2
Q

“Scleroderma Lung”

A 
1. Interstitial Lung Disease
A) nonspecific interstitial pneumonia (NSIP) pattern
B) interstitial fibrosis
2. Concentric arterial thickening
~67%get interstitial lung disease
~20% get pulmonary hypertension
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3
Q

IL-8

A

scleroderma lung, early, neutrophil attractant and activator

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4
Q

TNF

A

scleroderma lung, early, neutrophil attractant and promoter of neutrophil-endothelial binding

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5
Q

MIF1alpha

A

scleroderma lung, promoter of neutrophil chemotaxis

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6
Q

RANTES

A

scleroderma lung, acronym for regulated on activation normal T cell expressed and secreted
-T cell recruiter and activator

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7
Q

Endothelin-1

A

scleroderma lung, vasoconstrictor

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8
Q

TGF-beta

A

scleroderma lung, late, promoter of fibroplasia

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9
Q

Location of Scleroderma Lung

A

-favors bases, posterior & periphery

CT: ground-glass, then reticular infiltrates

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10
Q

Scleroderma Lung Micrograph

A
  • most common pattern of early disease is nonspecific interstitial pneumonia (NSIP) with lymphocytes and microphage infiltrating interstitium -fibroblasts + collagen = interstitial fibrosis
  • similar to UIP pattern, tell by temporal homogeneity (all areas at same stage)
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11
Q

Why distinguish NSIP “pattern” from NSIP?

A

different treatment

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12
Q

What stain to see systemic sclerosis?

A

Trichrome stain - extensive excess collagen stained blue

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13
Q

“Scleroderma lung” vascular disease

A
  • concentric thickening & fibrosis of small pulmonary arteries
  • causes pulmonary hypertension & cor pulmonale
  • can occur w/o interstitial lung disease (CREST, anti-centromere)
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14
Q

Systemic Sclerosis: Symptoms

A
  • Dyspnea (55%), increased work of breathing (stiff lungs) - sensed by respiratory muscle mechanoreceptors
  • Dry cough (<50%)
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15
Q

Systemic Sclerosis: Signs

A

-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

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16
Q

Systemic Sclerosis: Diagnosis

A
  • association of symptoms, signs and radiology of interstitial lung disease with skin, esophageal and renal manifestations of scleroderma and/or renal manifestations of scleroderma and/or anti-Scl70 (anti-DNA topoisomerase abs (40%)
  • restrictive pattern abnormalities due to reduced compliance and decreased diffusing capacity due to alveolar-capillary block or pulmonary hypertension on pulmonary function tests
  • Open lung biopsy if needed*
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17
Q

Systemic Sclerosis: Treatment

A

Cyclophosphamide NOT steroids

-steroids = scleroderma renal crisis

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18
Q

NSIP: Treatment

A

steroids

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19
Q

Systemic Sclerosis: Prognosis

A

mean: 12 years
Death rate 3.9%/year men
2.6%/year women
-cause of death, scleroderma lung disease (60%)

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20
Q

Lupus

A

1) Pleuritis
2) Acute lupus pneumonitis
3) nonspecific interstitial pneumonia
4) pulmonary vascular disease
- concentric arterial thickening
- pulmonary thromboembolism
5) Shrinking lung syndrome

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21
Q

Pleuritis

A

Lupus
-most common pleuropulmonary manifestation of lupus
20% at onset, 50% over time, 100% autopsy
-often asymptomatic, but sometimes have pleuritic chest pain, which can be recurrent or intractable

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22
Q

Lupus Pleuritis

A

fibrinous: +/- associated serosanguinous exudative pleural effusions
- few inflammatory cells in exudate
- ANA in pleural fluid

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23
Q

Fibrinous Pleuritis

A
  • partly organized by fibroblasts and densely adherent, but partly peeled off
  • pleural effusions of lupus usually small and bilateral
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24
Q

Pleuropulmonary disease of lupus

A

1) broader spectrum (more types)

2) more commonly acute than any other autoimmune connective tissue disease (collagen vascular disease)

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25
Q

Acute Lupus Pneumonitis

A

1) rare (2% of lupus patients)
2) actually a form of acute lung injury (diffuse alveolar damage)
3) can take form of diffuse hemorrhage

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26
Q

Acute Lupus Pneumonitis Micrograph/Disease Progression

A
  • alveolitis with loose fibrin exudate & lymphocytes & macrophages in airspaces and a few lymphocytes in interstitial
  • can be hemorrhagic
  • goes on to interstitial pneumonia & fibrosis
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27
Q

Acute Lupus Pneumonitis: Symptoms

A

dyspnea, fever, cough

28
Q

Acute Lupus Pneumonitis: Signs

A

fever, wet pulmonary crackles

29
Q

Acute Lupus Pneumonitis: CT

A

diffuse ground-glass opacification (+ pleural effusion, 50%)

30
Q

Acute Lupus Pneumonitis: Diagnosis

A

symptoms, + signs, + CT findings, + context (demographic + serology)

31
Q

Acute Lupus Pneumonitis: Treatment

A

steroid + immunosuppression

32
Q

Acute Lupus Pneumonitis: Prognosis

A

50% mortality

33
Q

Nonspecific interstitial pneumonia

A

~2% at onset
<5% over time
similar to NSIP without lupus

34
Q

Pulmonary Vascular Disease with Lupus

A
  • 10% of lupus patients have subclinical evidence of pulmonary hypertension on echocardiography
  • need to differentiate which of the 2 types a patient has, conc. on arterial thickening or thromboembolic disease
35
Q

Thromboembolic disease

A

-associated with “lupus anticoagulant” or anti-phospholipid antibodies

36
Q

Chronic Pulmonary Thromboembolic Disease

A
  • not specific for lupus

- pulmonary artery webs, strands of fibrous tissue spanning the lumen = old (organized) pulmonary emboli

37
Q

Shrinking Lung Syndrome with Lupus

A 
Rare, due to diaphragmatic weakness
Syndrome:
1) dyspnea
2) small lungs on x-ray
3) decreased diffusing capacity (DLCO)
4) restrictive pattern PFT abnormalities
-usually self limited
38
Q

Pleuropulmonary Disease in RA

A

-14% have “significant” disease, 44% subclinical
-3X more in males
-more common in smokers
Types:
1) pleuritis +/- pleural effusion
2) fibrosing interstitial lung disease
3) follicular bronchiolitis
4) obliterative bronchiolitis
5) organizing pneumonia
6) acure lung injury
7) pulmonary hemorrhage
8) rheumatoid nodules

39
Q

Pleuritis with fibrinous pleural effusion

A

20% RA patients have pleuritic pain
<5% pleural effusions
50% pleural disease at autopsy

40
Q

RA related disease on microscope

A
  • abundant lymphocytes and germinal centers
  • commonly involves the bronchioles
  • commonly involves pleura
41
Q

UIP Pattern fibrosing interstitial lung disease

A

Temporally Heterogeneous:

1) early levels of only inflammation
2) late areas of only fibrosis
3) intermediate areas with mix of inflammation, repair, fibrosis
- fibrotic lung remodeling that juxtaposes normal lung with honeycomb lung (end-stage)

42
Q

UIP Pattern fibrosing interstitial lung disease

Location

A

-most severe in periphery(subpleural) and lower lobes (basal lower lobes)

43
Q

Follicular bronchiolitis

A

-more common with RA
CT: peri-bronchial or centrilobular nodes and ground-glass opacities in bronchocentric distribution
-responds to steroids

44
Q

Obliterative Bronchiolitis

A
  • more common with RA
  • obstructive pattern PFTs
  • sometimes=superimposed Sjogren’s
  • does NOT respond to steroids
45
Q

Organizing Pneumonia

A
  • more common with RA

- present with acute onset, fever, alveolar infiltrates

46
Q

Interstitial Pneumonia plus acute lung injury

A

edema

hyaline membranes

47
Q

Interstitial Pneumonia plus acute lung injury

Micrograph

A
  • Rheumatoid nodule in lung with large area of necrosis
  • higher power showing rim of palisading histiocytes, basophilic debris and surrounding fibrosis, lymphocytes, macrophages and multinucleate giant cell
48
Q

Methotrexate

A

used to treat rheumatologic diseases (RA)

49
Q

Pneumonitis

A

adverse affect of methotrexate
-due to cytotoxicity causing diffuse alveolar damage or hypersensitivity causing chronic interstitial pneumonia (similar microscopic features)

50
Q

Methotrexate Pulmonary Toxicity

Early

A

not to bad, patchy lymphocytic interstitial infiltration

51
Q

Methotrexate Pulmonary Toxicity

Late

A

bad, fibrotic

52
Q

Methotrexate Pulmonary Toxicity

A
  • life-thretening (20% mortality)
  • responds to steroids
  • subacute (first 4 months)
  • dyspnea, cough, fever
  • bilateral pulmonary crackles
53
Q

Methotrexate Pulmonary Toxicity CT

A

bilateral, ground glass, reticular opacification

worse: coarser, denser, more reticular

54
Q

Pulmonary Toxicity of RA

A

1) acute chronic lung injury or chronic interstitial pneumonia
2) Penillamine: alveolar hemorrhage
3) Anti-tumor necrosis factor lpoagents, interstitial pneumonia

55
Q

Polymyosisit/dermatomyositis associated lung disease - Similarities to other CTD

A

-chronic
-NSIP
-dyspnea
-ground glass/reticular infiltrates
-restrictive pattern (PFT abnormalities, decreased DLCO)
-present with acute lung injury
Treat: steroids +/- immunosuppression

56
Q

Polymyosisit/dermatomyositis associated lung disease - Differences

A
  • occurs in 70% of patients
  • most common cause of death
  • ILD + organizing pneumonia
  • consolidation on CT scan (50%)
  • resp. failure from muscle weakness
  • anti-synthase syndrome: myositis + arthritis + lung disease
  • 12% patients have malignancy
57
Q

Sjogren Syndrome Similarities `

A
  • most common symptom is dyspnea (10%)
  • most common CT: ground glass lower lobe
  • is nonspecific interstitial pneumonia
58
Q

Sjogren Syndrome Differences

A
  • in 11% of patients
  • involves airways: submucosal glands
  • xerotrachea, xerobronchea
  • loss of submucosal gland secretion - relentless dry cough
  • bronchial hyper responsiveness like COPD
59
Q

Sjogren’s syndrome-associated NSIP

A

1) lymphoid follicles (germinal centers)
2) bronchiolocentric
3) more lymphocytes
4) macrophages/multinucleated giant cell

60
Q

Type of NSIP in Sjogren’s syndrome?

A

“cellular”

61
Q

Type of interstitial pneumonia in Sjogren’s syndrom?

A

“lymphocytic” “lymphoid”

-lots of lymphocytes that replicate and make mistakes - may have lymphoma

62
Q

Chronic Pheumonia: interstitial

A

Pneumocysits
Sarcoidosis
Toxoplasmosis

63
Q

Chronic Pheumonias: nodular

A 
TB
histoplasmosis
aspergillosis
cryptococcosis 
coccidioidomycosis
blastomycosis
64
Q

Acute lung disease in RA patient is?

A

Infectious

65
Q

Subacute or chronic dyspnea in RA patient?

A

rheumatologic

66
Q

Nodular lung disease in RA patient?

A

infectious or neoplastic

Musculoskeletal (19 decks)

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