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57 Cards in this Set

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Generally what you're looking for on CXR for interstitial lung disease
bilateral interstial opacities
alveorlar disease looks like what on CXR?
Fluffy, cotten-y,
ILD Clinical Features (4)
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)
ILD Physical Exam (4)
1) Bibasilar crackles
2) Clubbing
3) Signs of pulmonary hypertension
4) Signs of systematic involvement
Chest X-Ray: General Features
Bilateral interstitial changes, usually symmetric

Volume loss
Types of interstitial pattern (5)
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
classic ild
?
ild
?
What do Pulmonary function tests show in ILD?
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
What do Arterial blood gases show in ILD (early) ?
Early: Rest: near normal PO2 (generally low PCO2)
- Exercise: sharp drop in O2 sat
What do Arterial blood gases show in ILD (late) ?
Late: Rest: Hypoxemia worsened by exercise (PCO2 may rise to normal or elevated values as respiratory status worsens)
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
mononuclear
Pathology assoc with ILD
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
This is sarcoidosis. What's in the middle?
Granuloma
What can cause granulomatis inflammation?
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)
Causes of Pneumoconiosis
silica, asbestos, beryllium, coal
Causes of Hypersensitivity pneumonitis
thermophillic actinomyces, bacteria, amoebae, fungi
(farmer's lung)
Drugs/Poisons assoc with ILD
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
Connective Tissue diseases Associated with ILD
1. Rheumatoid arthritis
2. Progressive systemic sclerosis
3. Mixed connective tissue disease
4. Polymyositis / dermatomyositis
5. Systemic lupus erythematosus
6. Ankylosing spondylitis
7. Sjogrens
Idiopathic association with ILD
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
Vasculitides having the Lung Primarily involved:
Wegener’s granulomatosis
Lymphomatoid granulomatosis
Churg-Strauss vasculitis
Vasculitides having the Lung secondarily involved
Systemic leukocytoclastic vasculitis
Infectious associationws with ILD
Usually residue from widespread active
inflammation: mycobacterial, fungal, parasitic, Pneumocystis, viral
General Approach to the Patient With Interstitial Lung Disease
A. Establish diagnosis
B. Evaluate extent / severity of pulmonary and nonpulmonary involvement
C. Evaluate inflammatory activity (controversial)
D. Treatment
Diagnostic Evaluation of Interstitial Lung Disease
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
Classic ILD with lymphadenopathy - hilar adenopathy = sarcoidosis
?
Chest X-ray
Upper Lobe involvement Predominant:
A Ankylosing spondylitis
S Sarcoidosis
S Silicosis
E Eosinophilic granuloma
T Tuberculosis
Lower Lobe Predominant
B Bronchiectasis
A Asbestosis
D DIP / UIP / IPF
B Breast
A Aspiration
S Sarcoidosis
S Scleroderma
Mid-Lung Predominant
Sarcoidosis
Uremia
Congestive heart failure
Goodpasture’s
Pulmonary alveolar proteinosis
Pulmonary histology not needed for what disorders?
A. Collagen vascular
B. Asbestosis
When lung tissue is needed, it can be obtained via:
A. Transbronchial bx
B. Open bx / Video Assisted Thoracoscopy (VATS)– the gold standard
Transbronchial bx – useful if positive, especially in sarcoidosis, infections, tumor; not interpretable if normal or if shows "______"
“interstitial pneumonitis”
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
ruling out other diagnoses.
Interstitial Lung Diseases That can be Diagnosed by Transbronchial Biopsy (10)
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
Interstitial Lung Diseases that can be Diagnosed by Bronchoalveolar Lavage (4)
1. Pulmonary Alveolar Proteinosis
2. Pulmonary Hemorrhage Syndromes
3. Histiocytosis X
4. Infections
Interstitial Lung Diseases with Increased Polymorphonuclear Leukocytes on Bronchoalveolar Lavage (6)
1. Idiopathic Pulmonary Fibrosis
2. Collagen Vascular Disorders
3. Asbestosis
4. ARDS
5. BOOP
6. Infection
Interstitial Lung Diseases with Increased Lymphocytes on Bronchoalveolar Lavage (4)
1. Sarcoidosis
2. Hypersensitivity Pneumonitis
3. BOOP
4. Idiopathic Pulmonary Fibrosis
Esophageal manometrics is associated with what disease
(Scleroderma)
Collagen vascular serologies show what?
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)
IgG precipitating antibodies – positive in 90% of patients with acute ______ _______
hypersensitivity pneumonitis
ACE – abnormally high levels suggestive but not diagnostic of ______
sarcoidosis
Evaluation of Extent / Severity of Organ Involvement:
Pulmonary tests:
A. PFT with DL CO
B. Exercise ABG or O2 sat (oximetry)
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?
no
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 _________
fibrosis
Indices of Sarcoidosis
(5)
(1) ACE
(2) Lavage (% T lymphs, % OKT4 (+) lymphs)
(3) SPEP
(4) Gallium scan
(5) Lysozyme
Indices of Idiopathic pulmonary fibrosis (2)
(1) Gallium scan
(2) Lavage (% polys, % eos)
Treatment for asbestosis
none
Treatment for Eosinophillic granuloma
not effective
Treatment for Idiopathic pulmonary fibrosis, sarcoidosis, hypersensitivity pneumonitis
steroids still controversial as to who to treat and with how much;
cytotoxic agents even more controversial;
gamma interferon
Treatment of Wegener’s
cytotoxin + steroids;
imuran, bactrim in selected patients
Treatment of Goodpasture’s
cytoxan + plasmapheresis
O2 for patients with:
A. Polycythemia
B. PaO2 < _?_ (resting, exercise, or sleep)
55
most likely findings in moderate ILD
lung capacity decr
FEV1 percent normal
diff capac decr
most common blood gas finding in early mild ILD
mild resp alkalosis
no resting hypoxemia
oxy dest with exercise
no metabolic acidosis (distractor)
there are 2 type of granuloma
necrotizing (necrosis involved)
non-necrotizin
necrotizing disease?
TB
non-necrotizing granulomas diseases
sarcoid and autoimmune dis