Parenti 10-5-07 Interstitial Lung Disease

Front 1

Generally what you're looking for on CXR for interstitial lung disease

Back 1

bilateral interstial opacities

Front 2

alveorlar disease looks like what on CXR?

Back 2

Fluffy, cotten-y,

Front 3

ILD Clinical Features (4)

Back 3

Insidious onset of DOE and/or cough is most common.
Spontaneous pneumothorax (especially eosinophillic granuloma)
Manifestations of extrathoracic disease (sarcoidosis, collagen vascular)
Can present with systemic symptoms (fever, weight loss, arthralgias)

Front 4

ILD Physical Exam (4)

Back 4

1) Bibasilar crackles
2) Clubbing
3) Signs of pulmonary hypertension
4) Signs of systematic involvement

Front 5

Chest X-Ray: General Features

Back 5

Bilateral interstitial changes, usually symmetric

Volume loss

Front 6

Types of interstitial pattern (5)

Back 6

A. Ground glass
Diffuse clouding: discrete lesions cannot be detected
Seen with early stages of most ILD
B. Nodular
Often reflects blood stream dissemination
(miliary Tb, talcosis)
C. Reticular – networks of linear densities
D. Reticulonodular – combination of b and c
E. Honeycomb
Air-containing, cystic spaces 5-10 mm in diameter in vicinity of overt interstitial disease
Represents end-stage fibrotic lung of any etiology

Front 7

classic ild

Back 7

?

Front 8

ild

Back 8

?

Front 9

What do Pulmonary function tests show in ILD?

Back 9

A. Restrictive lung disease
B. Low diffusing capacity (DLCO); striking drop in oxygenation during exercise
C. Usually normal air flows (FEV1%)
D. May have obstructive component; small airways disease, superimposed bronchiectasis

Front 10

What do Arterial blood gases show in ILD (early) ?

Back 10

Early: Rest: near normal PO2 (generally low PCO2)
- Exercise: sharp drop in O2 sat

Front 11

What do Arterial blood gases show in ILD (late) ?

Back 11

Late: Rest: Hypoxemia worsened by exercise (PCO2 may rise to normal or elevated values as respiratory status worsens)

Front 12

Interstitial pneumonitis (alveolitis):
A. Usually mixed population of cells (mononuclear cell/polys) with _________ cell predominance
B. May have eosinophilic component
C. Granulomas may be present

Back 12

mononuclear

Front 13

Pathology assoc with ILD

Back 13

1) Interstitial pneumonitis (alveolitis)
2) Fibrosis
3) Bronchiectasis
4) As disease progresses, may see sequence of: interstitial pneumonitis→ pneumonitis plus fibrosis → severe fibrosis → honeycombing + bronchiectasis

Front 14

This is sarcoidosis. What's in the middle?

Back 14

Granuloma

Front 15

What can cause granulomatis inflammation?

Back 15

look it up (Crohn's disease, tuberculosis, Leprosy, sarcoidosis, berylliosis and syphilis. It is also a feature of Wegener's granulomatosis and Churg-Strauss syndrome, two related autoimmune disorders-per wikipedia)

Front 16

Causes of Pneumoconiosis

Back 16

silica, asbestos, beryllium, coal

Front 17

Causes of Hypersensitivity pneumonitis

Back 17

thermophillic actinomyces, bacteria, amoebae, fungi
(farmer's lung)

Front 18

Drugs/Poisons assoc with ILD

Back 18

1. Chemotherapeutic agents (bleomycin, busulfan, methotrexate, chlorambucil, melphalan, etc.)
2. Antibiotics (nitrofurantoin)
3. Gold
4. Methylsergide
5. Paraquat
6. Hexamethonium, mecamylamine, pentolinium, etc.
7. Talc
8. Hydrochlorothiazide
9. Cromolyn
10. Amiodarone
11. Cooking oil contaminated with rapseed oil

Front 19

Connective Tissue diseases Associated with ILD

Back 19

1. Rheumatoid arthritis
2. Progressive systemic sclerosis
3. Mixed connective tissue disease
4. Polymyositis / dermatomyositis
5. Systemic lupus erythematosus
6. Ankylosing spondylitis
7. Sjogrens

Front 20

Idiopathic association with ILD

Back 20

1. Sarcoidosis
2. Vasculitis (interstitial disease usually is minor component)
3. Histiocytosis X – Eosinophilic granuloma
4. Idiopathic pulmonary fibrosis
5. Idiopathic pulmonary hemosiderosis
6. Chronic pulmonary alveolar proteinosis
7. Tuberous sclerosis
8. Familial pulmonary fibrosis

Front 21

Vasculitides having the Lung Primarily involved:

Back 21

Wegener’s granulomatosis
Lymphomatoid granulomatosis
Churg-Strauss vasculitis

Front 22

Vasculitides having the Lung secondarily involved

Back 22

Systemic leukocytoclastic vasculitis

Front 23

Infectious associationws with ILD

Back 23

Usually residue from widespread active
inflammation: mycobacterial, fungal, parasitic, Pneumocystis, viral

Front 24

General Approach to the Patient With Interstitial Lung Disease

Back 24

A. Establish diagnosis
B. Evaluate extent / severity of pulmonary and nonpulmonary involvement
C. Evaluate inflammatory activity (controversial)
D. Treatment

Front 25

Diagnostic Evaluation of Interstitial Lung Disease

Back 25

1. History and Physical
2. Chest x- ray
3. CT chest (high resolution)
4. Pulmonary Function Tests
5. Arterial blood gas, exercise oximetry
6. Various Serology
7. Gallium Scan
8. Bronchoscopy with Bronchoalveolar Lavage and Transbronchial Biopsy
9. Open Lung Biopsy

Front 26

Classic ILD with lymphadenopathy - hilar adenopathy = sarcoidosis

Back 26

?

Front 27

Chest X-ray
Upper Lobe involvement Predominant:

Back 27

A Ankylosing spondylitis
S Sarcoidosis
S Silicosis
E Eosinophilic granuloma
T Tuberculosis

Front 28

Lower Lobe Predominant

Back 28

B Bronchiectasis
A Asbestosis
D DIP / UIP / IPF
B Breast
A Aspiration
S Sarcoidosis
S Scleroderma

Front 29

Mid-Lung Predominant

Back 29

Sarcoidosis
Uremia
Congestive heart failure
Goodpasture’s
Pulmonary alveolar proteinosis

Front 30

Pulmonary histology not needed for what disorders?

Back 30

A. Collagen vascular
B. Asbestosis

Front 31

When lung tissue is needed, it can be obtained via:

Back 31

A. Transbronchial bx
B. Open bx / Video Assisted Thoracoscopy (VATS)– the gold standard

Front 32

Transbronchial bx – useful if positive, especially in sarcoidosis, infections, tumor; not interpretable if normal or if shows "______"

Back 32

“interstitial pneumonitis”

Front 33

Open bx / Video Assisted Thoracoscopy (VATS)– the gold standard; however, still is often only compatible with a diagnosis and most helpful in __________.
Tissue should be obtained from several areas within lung, including obviously involved regions, and areas that appear relatively normal

Back 33

ruling out other diagnoses.

Front 34

Interstitial Lung Diseases That can be Diagnosed by Transbronchial Biopsy (10)

Back 34

1. Sarcoidosis
2. Inorganic Dust Diseases
3. Hypersensitivity Pneumonitis
4. Histiocytosis X
5. Pulmonary Hemorrhage Syndromes
6. Eosinophilic Pneumonia
7. Drug – induced Interstitial Disease
8. Lymphangioleiomyomatosis
9. Infections
10. Tumors

Front 35

Interstitial Lung Diseases that can be Diagnosed by Bronchoalveolar Lavage (4)

Back 35

1. Pulmonary Alveolar Proteinosis
2. Pulmonary Hemorrhage Syndromes
3. Histiocytosis X
4. Infections

Front 36

Interstitial Lung Diseases with Increased Polymorphonuclear Leukocytes on Bronchoalveolar Lavage (6)

Back 36

1. Idiopathic Pulmonary Fibrosis
2. Collagen Vascular Disorders
3. Asbestosis
4. ARDS
5. BOOP
6. Infection

Front 37

Interstitial Lung Diseases with Increased Lymphocytes on Bronchoalveolar Lavage (4)

Back 37

1. Sarcoidosis
2. Hypersensitivity Pneumonitis
3. BOOP
4. Idiopathic Pulmonary Fibrosis

Front 38

Esophageal manometrics is associated with what disease

Back 38

(Scleroderma)

Front 39

Collagen vascular serologies show what?

Back 39

A. sed rate, ANA, rheumatoid factor
B. anti Scl – 70 , anti centromere antibody
C. ANCA (anti neutrophil cytoplasmic antibody)
D. anti Jo, anti PM 1
E. antiglomerular basement membrane antibody (Goodpasteurs)

Front 40

IgG precipitating antibodies – positive in 90% of patients with acute ______ _______

Back 40

hypersensitivity pneumonitis

Front 41

ACE – abnormally high levels suggestive but not diagnostic of ______

Back 41

sarcoidosis

Front 42

Evaluation of Extent / Severity of Organ Involvement:
Pulmonary tests:

Back 42

A. PFT with DL CO
B. Exercise ABG or O2 sat (oximetry)

Front 43

The concept of alveolitis
A. Inflammation of the lower respiratory tract precedes and causes subsequent fibrosis
B. The symptoms and physiologic derangements of ILD can result from inflammation or fibrosis. Are those due to fibrosis are reversible?

Back 43

no

Front 44

Quantification of inflammatory activity may provide an index of:
(1) Amount of reversibility in a patient’s defect
(2) Risk of a patient subsequently developing pulmonary _________

Back 44

fibrosis

Front 45

Indices of Sarcoidosis
(5)

Back 45

(1) ACE
(2) Lavage (% T lymphs, % OKT4 (+) lymphs)
(3) SPEP
(4) Gallium scan
(5) Lysozyme

Front 46

Indices of Idiopathic pulmonary fibrosis (2)

Back 46

(1) Gallium scan
(2) Lavage (% polys, % eos)

Front 47

Treatment for asbestosis

Back 47

none

Front 48

Treatment for Eosinophillic granuloma

Back 48

not effective

Front 49

Treatment for Idiopathic pulmonary fibrosis, sarcoidosis, hypersensitivity pneumonitis

Back 49

steroids still controversial as to who to treat and with how much;
cytotoxic agents even more controversial;
gamma interferon

Front 50

Treatment of Wegener’s

Back 50

cytotoxin + steroids;
imuran, bactrim in selected patients

Front 51

Treatment of Goodpasture’s

Back 51

cytoxan + plasmapheresis

Front 52

O2 for patients with:
A. Polycythemia
B. PaO2 < _?_ (resting, exercise, or sleep)

Back 52

55

Front 53

most likely findings in moderate ILD

Back 53

lung capacity decr
FEV1 percent normal
diff capac decr

Front 54

most common blood gas finding in early mild ILD

Back 54

mild resp alkalosis
no resting hypoxemia
oxy dest with exercise
no metabolic acidosis (distractor)

Front 55

there are 2 type of granuloma

Back 55

necrotizing (necrosis involved)
non-necrotizin

Front 56

necrotizing disease?

Back 56

TB

Front 57

non-necrotizing granulomas diseases

Back 57

sarcoid and autoimmune dis