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Kendig > Pulmonary Involvement In Systemic Inflammatory Disease > Flashcards

Pulmonary Involvement In Systemic Inflammatory Disease Flashcards

1
Q

Life-threatening complication of JIA

A

Macrophage activation syndrome

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2
Q

Pulmonary involvement in JIA

A

Pleuritis, usually with pericarditis
Pulmonary hemosiderosis
Lymphoid folllicular bronchiolitis
Lymphocytic interstitial fibrosis
Alveolar proteinosis

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3
Q

Pulmonary manifestations of MAS

A

Pulmonary hemorrhage
Pulmonary edema
Pneumonitis

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4
Q

PFT in JIA

A

Restrictive > obstructive
Varying reductions in DLCO

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5
Q

Treatment of JIA

A
  1. NSAIDs; CS if unresponsive
  2. Methotrexate
  3. TNFa inhibitors (etanercept, adalimumab, infliximab)

Others: IL-1 antagonists, IL-6 antagonist (tocilizumab), cyclosporine

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6
Q

Prognosis of JIA

A

Low mortality
Acute pleuritis responds well without sequelae
High mortality in PAH, ILD, AP

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7
Q

Pulmonary involvement in SLE

A

Pleuritis with pleural effusion
Pulmonary infections
Inflammatory pulmonary lesions

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8
Q

Pleural effusion in SLE

A

Exudative

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9
Q

PFT in SLE

A

Slow, progressively restrictive pattern which may improve or stabilize

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10
Q

Histopathology in SLE

A

Alveolar wall thickening, interstitial fibrosis and infiltrates, deposits of immunoglobulin and complement

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11
Q

Rare complications of SLE

A

Pulmonary hemorrhage
Pulmonary hypertension
Shrinking lung syndrome

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12
Q

Common infections in pulmonary hemorrhage in SLE

A

Aspergillosis
Pseudomonas
CMV

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13
Q

Risk factor for pulmonary hypertension in SLE

A

Lupus anticoagulant

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14
Q

Treatment of SLE

A

Pleural effusion - corticosteroid
Pulmonary hemorrhage - MPPT, CS, cyclophosphamide, plasmapharesis

Others: hydroxychloroquine, rituximab

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15
Q

Most common cause of death in SLE

A

Infection

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16
Q

Associated with ILD in JDM in adults

A

Anti-Jo-1
Antisynthetase antibodies

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17
Q

Pulmonary involvement in JDM

A

ILD - can be rapidly progressive
Pneumomediastinum
Aspiration pneumonia
Hypoventilation

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18
Q

Most common presenting symptoms of ILD in JDM

A

Cough, dyspnea

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19
Q

PFTs in JDM

A

Restrictive - decreased lung volumes, normal or elevated FEV1/FVC
Reduced DLCO

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20
Q

HRCT in JDM

A

Irregular linear opacities with areas of consolidation and GGO (active inflammation)
Honeycombing is uncommon

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21
Q

Treatment of JDM

A

Corticosteroid, MPPT
Methotrexate
Cyclosporine + CS
IVIg
Rituximab

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22
Q

Pulmonary involvement in SSc

A

ILD
PAH
Cardiomyopathy

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23
Q

Mediator of vascular changes in SSc

A

Endothelin-1

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24
Q

Radiographic findings in SSc

A

GGO
Reticular pattern
Traction bronchiectasis
Honeycombing
Subpleural micronodules

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25
Q

DLCO in SSc

A

Reduced due to ILD or PAH

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26
Q

PFTs in SSc

A

Restrictive due to limited chest wall expansion, lung volume loss, pulmonary fibrosis

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27
Q

Associated with ILD in SSc

A

KL-6

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28
Q

Treatment for SSc

A

Cyclophosphamide
Lung transplantation
Stem cell transplantation

Others: MMF, azathioprine, rituximab

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29
Q

MCTD clinical features are a combination of:

A

SSc, SLE, JDM

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30
Q

Antibodies associated with MCTD

A

Anti-U1 RNP

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31
Q

Differentiates MCTD from SLE

A

Absence of neurologic or renal disease in MCTD

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32
Q

Pulmonary involvement in MCTD

A

Pulmonary fibrosis
Pulmonary effusion
PAH

Less common:
Thromboembolic disease
Pulmonary hemorrhage
Diaphragmatic dysfunction
Aspiration pneumonitis

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33
Q

Treatment of MCTD

A

Glucocorticoids
NSAIDs
Hydroxychloroquine

Others:
Nifedipine for Reynaud phenomenon
Methotrexate
Cytotoxics

34
Q

Treatment of PAH in MCTD

A

Prostacycline analogues
Endothelin receptor antagonists
PDE5 inhibitors

35
Q

Prognosis of MCTD

A

Incurable
Deaths are often due to rapid-onset PAH and infections

36
Q

Pathology of sarcoidosis

A

Noncaseating epithelioid cell granulomas with predilection for thoracic lymph nodes and lung tissue

37
Q

Long-term morbidities of sarcoidosis

A

Pulmonary fibrosis
Uveitis

38
Q

Pathogenesis of sarcoidosis

A

Exaggerated TH-1 immune response to unidentified antigen in genetically susceptible individuals leading to granuloma formation

39
Q

Hallmark of sarcoidosis

A

Granulomatous lesions, noncaseating, located in perilymphatic areas, subpleural soace, and perilobular spaces

40
Q

Fate of granulomatous lesions

A

Heal with preservation of lung parenchyma or produce fibrotic scar tissue

41
Q

Acute arthritis, BHL, erythema nodosum

A

Löfgren Syndrome

42
Q

Pulmonary involvement in sarcoidosis

A

Commonly affects intrathoracic LN and parenchyma
Dyspnea, wheezing, cough, chest pain

43
Q

Staging of BHL in sarcoidosis

A

0: normal
1: BHL
2: BHL + parenchymal infiltrates
3. Parenchymal infiltrates without BHL
4: pulmonary fibrosis

44
Q

HRCT of sarcoidosis

A

Widespread micronodules and nodules in perilymphatic/peribronchovascular distribution
Nodular pleural thickening
Thickening of interlobular septae
Hilar, mediastinal LAD
Pulmonary fibrosis with honeycombing if long-standing

45
Q

PFT and DLCO of sarcoidosis

A

Restrictive > obstructive
Normal PFT in St. 0 or 1
Reduced DLCO

46
Q

Airway involvement in sarcoidosis

A

Waxy yellow mucosal nodules
Bronchial stenosis
Airway hyperreactivity

47
Q

Complications of sarcoidosis

A

Pulmonary fibrosis
End-stage lung disease
Bronchiectasis
Chronic infection
Aspergilloma

48
Q

Diagnosis of sarcoidosis

A

Tissue biopsy with noncaseating granulomas + clinical criteria
Biopsy NOT NEEDED for Löfgren

49
Q

BAL profile in sarcoidosis

A

Lymphocytic

50
Q

Treatment of sarcoidosis

A

Corticosteroids for 12-18 months
Methotrexate
Hydroxychloroquine

51
Q

Prognosis of sarcoidosis

A

Spontaneous recovery in 2/3
Progressive pulmonary fibrosis and end-stage CLD if St. 3 on presentation
Excellent prognosis for Löfgren

52
Q

Large- or medium-sized vasculitis causes:

A

Arterial insufficiency (infarction, necrosis, end organ dysfunction)

53
Q

Small-sized vasculitis causes:

A

Leakage of blood into tissue (DAH)

54
Q

Small-vessel vasculitides with frequent pulmonary involvement (ANCA-associated vasculitis)

A

GPA
MPA
EGPA
IPC

55
Q

Features of EGPA

A

Severe atopic asthma and AR

56
Q

Most common vasculitis syndrome presenting to pulmonologist

A

GPA

57
Q

Etiology of GPA

A

Autoimmune (universal presence of ANCA, response to immunosuppressive therapy)

58
Q

Pathogenesis of GPA

A

ANCA are directed against PR-3-ANCA, causing neutrophil activation and interaction with endothelium. Neutrophils migrate through the endothelium and degranulate, releasing toxic products and causing tissue damage. T-helper and B cells assist in autoantibody reaction.

59
Q

Histopathology of ANCA-associated vasculitis

A

Necrotizing vasculitis of small blood vessels WITHOUT immune complex deposition

60
Q

Unique about GPA among AAV

A

Anti-PR-3-ANCA
Granuloma

61
Q

Triad of GPA

A

Upper airway
Lower respiratory tract
Renal

62
Q

Presenting pulmonary symptoms of GPA

A

Dyspnea, chronic cough
Hemoptysis (necrotizing mucosal airway involvement)
Stridor and hoarseness (SG stenosis)

63
Q

Upper airway findings in GPA

A

Saddle-nose deformity

64
Q

Most common CXR finding

A

Nodules with fixed infiltrates

Others:
Cavitation
Mediastinal LAD
Pleural effusion
Pneumothorax

65
Q

Chest CT findings in GPA

A

Nodules (multiple, >5mm, cavitating in 17%)
GGO
Air space consolidations (hemorrhage)

66
Q

PFTs in GPA

A

Depends on tissues involved

67
Q

Bronchoscopy findings in GPA

A

Mucosal erythema
Edema
Ulceration
Hemorrhage
Cobblestoning
Nodules
Polyps
Submucosal tunnelling
Synechial bands
Stenosis

68
Q

Diagnosis of GPA

A

Clinical findings (e.g. pulmorenal syndrome)
Serology (anti-PR-3-ANCA)
Histopathology (pauci-immune granulomatous inflammation of small/medium-sized vessels)

69
Q

Treatment of GPA

A

Glucocorticoids, cyclophosphamide
Rituximab if severe

Maintenance:
Methotrexate
Azathioprine

70
Q

Treatment of GPA obstructive airway lesions

A

Intralesional steroid
Endoscopic dilatation
Stenting
Tracheostomy

71
Q

Prognosis of GPA

A

Most will respond but will relapse

72
Q

Pulmonary involvement of Sjogren’s sclerosis

A

Sicca cough
Small airway obstruction
Airway hyperreactivity
Follicular bronchiolitis
ILD/LIP

73
Q

Treatment of SS

A

Steroid
Cyclophosphamide
Infliximab

74
Q

DLCO in SS

A

Reduction that worsens during active disease

75
Q

Single most common disorder in SS in adults

A

Bronchiectasis

76
Q

Most common presentation for ILD

A

Slowly progressive dyspnea with or without dry cough

77
Q

HRCT correlations

A

GGO: parenchymal lung disease
Peribronchovascular changes and air trapping: airways disease
Pleural thickening with effusion: pleural inflammation

78
Q

DLCO in chest wall restriction

A

Reserved until with severe loss of volume

79
Q

PFT in bronchiectasis

A

Obstructive

80
Q

DLCO in DAH

A

Increased if hemorrhage is recent

Kendig (8 decks)

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