Pulmonary Involvement In Systemic Inflammatory Disease Flashcards

1
Q

Life-threatening complication of JIA

A

Macrophage activation syndrome

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

Pulmonary involvement in JIA

A

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

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

Pulmonary manifestations of MAS

A

Pulmonary hemorrhage
Pulmonary edema
Pneumonitis

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

PFT in JIA

A

Restrictive > obstructive
Varying reductions in DLCO

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

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

Prognosis of JIA

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Pulmonary involvement in SLE

A

Pleuritis with pleural effusion
Pulmonary infections
Inflammatory pulmonary lesions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Pleural effusion in SLE

A

Exudative

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

PFT in SLE

A

Slow, progressively restrictive pattern which may improve or stabilize

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Histopathology in SLE

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Rare complications of SLE

A

Pulmonary hemorrhage
Pulmonary hypertension
Shrinking lung syndrome

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

Common infections in pulmonary hemorrhage in SLE

A

Aspergillosis
Pseudomonas
CMV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Risk factor for pulmonary hypertension in SLE

A

Lupus anticoagulant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Treatment of SLE

A

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

Others: hydroxychloroquine, rituximab

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

Most common cause of death in SLE

A

Infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Associated with ILD in JDM in adults

A

Anti-Jo-1
Antisynthetase antibodies

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

Pulmonary involvement in JDM

A

ILD - can be rapidly progressive
Pneumomediastinum
Aspiration pneumonia
Hypoventilation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Most common presenting symptoms of ILD in JDM

A

Cough, dyspnea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

PFTs in JDM

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

HRCT in JDM

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Treatment of JDM

A

Corticosteroid, MPPT
Methotrexate
Cyclosporine + CS
IVIg
Rituximab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Pulmonary involvement in SSc

A

ILD
PAH
Cardiomyopathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Mediator of vascular changes in SSc

A

Endothelin-1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Radiographic findings in SSc

A

GGO
Reticular pattern
Traction bronchiectasis
Honeycombing
Subpleural micronodules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
DLCO in SSc
Reduced due to ILD or PAH
26
PFTs in SSc
Restrictive due to limited chest wall expansion, lung volume loss, pulmonary fibrosis
27
Associated with ILD in SSc
KL-6
28
Treatment for SSc
Cyclophosphamide Lung transplantation Stem cell transplantation Others: MMF, azathioprine, rituximab
29
MCTD clinical features are a combination of:
SSc, SLE, JDM
30
Antibodies associated with MCTD
Anti-U1 RNP
31
Differentiates MCTD from SLE
Absence of neurologic or renal disease in MCTD
32
Pulmonary involvement in MCTD
Pulmonary fibrosis Pulmonary effusion PAH Less common: Thromboembolic disease Pulmonary hemorrhage Diaphragmatic dysfunction Aspiration pneumonitis
33
Treatment of MCTD
Glucocorticoids NSAIDs Hydroxychloroquine Others: Nifedipine for Reynaud phenomenon Methotrexate Cytotoxics
34
Treatment of PAH in MCTD
Prostacycline analogues Endothelin receptor antagonists PDE5 inhibitors
35
Prognosis of MCTD
Incurable Deaths are often due to rapid-onset PAH and infections
36
Pathology of sarcoidosis
Noncaseating epithelioid cell granulomas with predilection for thoracic lymph nodes and lung tissue
37
Long-term morbidities of sarcoidosis
Pulmonary fibrosis Uveitis
38
Pathogenesis of sarcoidosis
Exaggerated TH-1 immune response to unidentified antigen in genetically susceptible individuals leading to granuloma formation
39
Hallmark of sarcoidosis
Granulomatous lesions, noncaseating, located in perilymphatic areas, subpleural soace, and perilobular spaces
40
Fate of granulomatous lesions
Heal with preservation of lung parenchyma or produce fibrotic scar tissue
41
Acute arthritis, BHL, erythema nodosum
Löfgren Syndrome
42
Pulmonary involvement in sarcoidosis
Commonly affects intrathoracic LN and parenchyma Dyspnea, wheezing, cough, chest pain
43
Staging of BHL in sarcoidosis
0: normal 1: BHL 2: BHL + parenchymal infiltrates 3. Parenchymal infiltrates without BHL 4: pulmonary fibrosis
44
HRCT of sarcoidosis
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
PFT and DLCO of sarcoidosis
Restrictive > obstructive Normal PFT in St. 0 or 1 Reduced DLCO
46
Airway involvement in sarcoidosis
Waxy yellow mucosal nodules Bronchial stenosis Airway hyperreactivity
47
Complications of sarcoidosis
Pulmonary fibrosis End-stage lung disease Bronchiectasis Chronic infection Aspergilloma
48
Diagnosis of sarcoidosis
Tissue biopsy with noncaseating granulomas + clinical criteria Biopsy NOT NEEDED for Löfgren
49
BAL profile in sarcoidosis
Lymphocytic
50
Treatment of sarcoidosis
Corticosteroids for 12-18 months Methotrexate Hydroxychloroquine
51
Prognosis of sarcoidosis
Spontaneous recovery in 2/3 Progressive pulmonary fibrosis and end-stage CLD if St. 3 on presentation Excellent prognosis for Löfgren
52
Large- or medium-sized vasculitis causes:
Arterial insufficiency (infarction, necrosis, end organ dysfunction)
53
Small-sized vasculitis causes:
Leakage of blood into tissue (DAH)
54
Small-vessel vasculitides with frequent pulmonary involvement (ANCA-associated vasculitis)
GPA MPA EGPA IPC
55
Features of EGPA
Severe atopic asthma and AR
56
Most common vasculitis syndrome presenting to pulmonologist
GPA
57
Etiology of GPA
Autoimmune (universal presence of ANCA, response to immunosuppressive therapy)
58
Pathogenesis of GPA
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
Histopathology of ANCA-associated vasculitis
Necrotizing vasculitis of small blood vessels WITHOUT immune complex deposition
60
Unique about GPA among AAV
Anti-PR-3-ANCA Granuloma
61
Triad of GPA
Upper airway Lower respiratory tract Renal
62
Presenting pulmonary symptoms of GPA
Dyspnea, chronic cough Hemoptysis (necrotizing mucosal airway involvement) Stridor and hoarseness (SG stenosis)
63
Upper airway findings in GPA
Saddle-nose deformity
64
Most common CXR finding
Nodules with fixed infiltrates Others: Cavitation Mediastinal LAD Pleural effusion Pneumothorax
65
Chest CT findings in GPA
Nodules (multiple, >5mm, cavitating in 17%) GGO Air space consolidations (hemorrhage)
66
PFTs in GPA
Depends on tissues involved
67
Bronchoscopy findings in GPA
Mucosal erythema Edema Ulceration Hemorrhage Cobblestoning Nodules Polyps Submucosal tunnelling Synechial bands Stenosis
68
Diagnosis of GPA
Clinical findings (e.g. pulmorenal syndrome) Serology (anti-PR-3-ANCA) Histopathology (pauci-immune granulomatous inflammation of small/medium-sized vessels)
69
Treatment of GPA
Glucocorticoids, cyclophosphamide Rituximab if severe Maintenance: Methotrexate Azathioprine
70
Treatment of GPA obstructive airway lesions
Intralesional steroid Endoscopic dilatation Stenting Tracheostomy
71
Prognosis of GPA
Most will respond but will relapse
72
Pulmonary involvement of Sjogren’s sclerosis
Sicca cough Small airway obstruction Airway hyperreactivity Follicular bronchiolitis ILD/LIP
73
Treatment of SS
Steroid Cyclophosphamide Infliximab
74
DLCO in SS
Reduction that worsens during active disease
75
Single most common disorder in SS in adults
Bronchiectasis
76
Most common presentation for ILD
Slowly progressive dyspnea with or without dry cough
77
HRCT correlations
GGO: parenchymal lung disease Peribronchovascular changes and air trapping: airways disease Pleural thickening with effusion: pleural inflammation
78
DLCO in chest wall restriction
Reserved until with severe loss of volume
79
PFT in bronchiectasis
Obstructive
80
DLCO in DAH
Increased if hemorrhage is recent