L13 Interstitial Lung disease Flashcards
(75 cards)
1
Q
Diffuse Parenchymal Lung Disease aka
A
Interstitial lung disease
2
Q
honeycombing
A
clustered cystic air spaces
3
Q
Interstitial lung disease pathophysiology
A
Extension distortion of the airway and alveolar compartment along with the interstitium causing progressive scarring of lung tissue surrounding the alveoli
Areas of fibrosis alternate with areas of normal lung
Honeycombing
4
Q
Causes of Interstitial lung disease
A
Idiopathic
Autoimmune
Exposure
Drugs
5
Q
exposure causes
A
asbestos, silica, bat/bird droppings, radiation, hot tubs
6
Q
drug causes
A
amiodarone propranolol nitrofurantoin methotrexate rituximab
7
Q
autoimmune causes
A
dermatomyositis,RA, scleroderma, sarcoidosis, sjogrens
8
Q
Interstitial lung disease is ______ so the treatment goal is to ______
A
IRREVERSIBLE
prevent progression/alleviate sx
9
Q
what symptoms are uncommon in Interstitial lung disease
A
wheezing, chest pain
10
Q
main sx of Interstitial lung disease
A
Progressive dyspnea on exertion
Persistent non-productive cough
11
Q
extra-pulmonary symptoms may indicate
A
connective tissue disease→ musculoskeletal pain, weakness, joint pain/swelling, fevers, dry eyes/mouth
12
Q
heard on lung exam of a patient with Interstitial lung disease
A
velcro crackles at bases
inspiratory squeaks
13
Q
late stage Interstitial lung disease signs
A
cyanosis, digital clubbing cor pulmonale (middle/late)
14
Q
Extrapulmonary manifestations: erythema nodosum
A
Sarcoidosis
15
Q
Extrapulmonary manifestations: gottron’s papules
A
Dermatomyositis
16
Q
highest yield for noninvasive test to diagnose Interstitial lung disease
A
High resolution chest CT (HRCT)
17
Q
Gold standard to diagnose Interstitial lung disease
A
Tissue biopsy
18
Q
findings on CXR that could indicate Interstitial lung disease
A
Ground glass appearance early, nonspecific
Reticular “netlike” (most common), nodular, or mixed pattern of opacities
Honeycombing: small cystic spaces: poor prognosis
19
Q
reticular opacities are
A
small irregular opacities
20
Q
Micronodular pattern is
A
small rounded opacities
21
Q
HRCT: reticular opacities
A
IPF
Asbestosis
22
Q
HRCT: ground glass attenuation
A
Drug toxicity
Respiratory bronchiolitis
23
Q
HRCT: upper/central lobes
A
Sarcoidosis
24
Q
Serologic studies to rule out subclinical autoimmune disease
A
ANA, RF
25
when to evaluate for vasculitis and how
pulmonary hemorrhage or suspicious systemic symptoms
| Antineutrophil cytoplasmic antibodies (ANCA)
26
signs of restrictive disease on PFTs
Decreased Total lung capacity
Decreased FEV1, FVC
Normal/increased FEV1/FVC
27
PFTs obstructive disease
Increased TLCC
Normal FVC
Decreased FEV1
Decreased FEV1/FVC ratio
28
If PFTs indicate restrictive disease, obtain
DLCO: low=ILD
29
ILD with obstructive pattern
sarcoidosis
30
Extension of bronchoscopy that allows sampling→ cell counts, culture (atypical/typical), cytology (identify maligancy)
Bronchoalveolar lavage
31
Is bronchoalveolar lavage typically performed with HRCT findings consistent with IPF
no
32
gold standard for diagnosis
lung biopsy
33
contraindication for lung biopsy
honeycombing
34
Transbronchial biopsy is a broncoscope + biopsy used to assess ____
central locations
| NOT periphery
35
2 types of surgical lung biopsies
1. Video assisted thoracoscopic surgery: 2 incisions in lateral chest wall
2. Thoracotomy: 5-6 cm incision
36
Bronchoscope used to evaluated hilar and mediastinal lymph nodes
useful if sarcoid suspected
Endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA)
37
2 biopsy methods that can be done in conjunction if a different scope is passed
Endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA)
-and-
Transbronchial lung biopsy
38
UIP=“Usual interstitial pneumonia”
Idiopathic pulmonary fibrosis
| Abestosis
39
most common interstitial lung disease
Idiopathic pulmonary fibrosis
40
what is HRCT used for in idiopathic pulmonary fibrosis
Determine if: probable/indeterminate UIP pattern, alternative diagnosis→ Bronchoalveolar lavage/lung biopsy
41
treatment for idiopathic pulmonary fibrosis
Treatment for GERD
| Nintedanib: tyrosine kinase inhibitor
42
Nintedanib is a _____ and works by
tyrosine kinase inhibitor
| downregulation of production of growth factors and procollagens
43
sarcoidosis extrapulmonary findings
erthema nodosum
lupus pernio
granulomatous uveitis
arthralgias
44
Non-caseating granulomas
sarcoidosis
→ secrete 1,25 dihydroxyvitamin D→ serum Ca++ elevated
→ secrete ACE
45
labs of sarcoidosis
Elevated serum ACE +/- elevated serum calcium, hypercalciuria
Elevated alkaline phosphatase
46
lupus pernio
idk some weird nose nodule
| sarcoidosis
47
CXR staging of sarcoidosis
0. Normal
1. Hilar adenopathy
2. Hilar adenopathy + diffuse infiltrates
3. Only diffuses parenchymal infiltrates
4. Pulmonary fibrosis
48
when to treat sarcoidosis
stage 2 and symptomatic
49
Sarcoidosis tx
high dose corticosteroids + PJP prophylaxis
methotrexate (alternative)
chloroquine/hydroxychloroquine: cutaneous lesions, neuro sx, hypercalcemia
topical corticosteroids for ocular disease
lung transplant: stage IV
50
most common CXR pattern of sarcoidosis LAD
bilateral symmetrical hilar and right paratracheal mediastinal adenopathy
51
inhalation and deposition of mineral dust
pneumoconiosis: asbestosis, silicosis, coal worker's
52
Chronic simple silicosis
10-12 years exposure
+/- asymptomatic
Nonprogressive once exposure eliminated
Hilar node calcification: eggshell pattern
53
Chronic complicated silicosis
> 20 years exposure
Progression even after exposure eliminated
Tachypnea, prolonged expiration, rhonchi, wheezing, rales
Advanced disease: cyanosis, cor pulmonale
54
silicosis classic CXR finding
eggshell calcifications
55
asbestosis presentation
15-20 years after exposure, dose dependent
40-75 year old males
Smoking increases risk of bronchogenic cancer
Presentation: Insidious onset, dyspnea, reduced exercise tolerance, chest discomfort, end inspiratory rales
Digital clubbing which does not correlate with severity
56
asbestosis imaging
HRCT: reticular opacities
Restrictive pattern
CXR: opacities in lower lungs, thickened pleura, pleural plaques
Open-lung biopsy not required but is definitive diagnosis: fibrosis, asbestos bodies through light microscopy
57
reliable indicator of asbestosis on CXR, not always present
calcified pleural plaques in diaphragmatic pleura of the 6th-9th ribs
58
asbestosis treatment
NO immunotherapy drugs or steroids alter disease course
| basically prevent exposure and treat symptoms
59
asbestosis has a high risk of
Cancer
risk increased by smoking→ bronchogenic carcinoma
→ cancers of upper respiratory tract, esophagus, biliary system, kidneys
60
Mesothelioma
form of cancer almost always associated with asbestos exposure, even short term (1-2) years
Cancer develops in mesothelium: protective lining covering organs: pleura (most common), peritoneum, pericardium
Not caused by smoking, poor prognosis
61
granulomatosis with polyangiitis aka
“ANCA-associated vasculitis”
62
granulomatosis with polyangiitis
Multisystem autoimmune disease
Systemic vasculitis of small/medium vessels
Necrotizing granulomas of upper and lower respiratory tracts
RARE, 35-55 year old northern europeans
Relapse common
63
granulomatosis with polyangiitis presentation
recurrent respiratory infections, constitutional symptoms (fever, weight loss, night sweats, anorexia, fatigue/lethargy)
Ocular: conjunctivitis, episcleritis, uveitis, RAO
ENT: chronic sinusitis, rhinitis, epistaxis, saddle nose deformity
Pulmonary: infiltrates, cough, hemoptysis, dyspnea, stridor
Renal: failure, erythrocyte casts
Peripheral nervous system: cranial nerve palsies, sensorimotor polyneuropathy
Skin: palpable purpura, skin ulcers, necrotic, blistering purpura
Cardiac: pericarditis, coronary vasculitis
Polyarticular arthralgias
64
specific granulomatosis with polyangiitis findings that distinguish it
saddle nose deformity
infiltrates of CXR
a million systemic symptoms of *vasculitis*
(+) C-ANCA
CT: stellate shaped peripheral pulmonary arteries= “vasculitis sign”
65
granulomatosis with polyangiitis lab findings
(+) C-ANCA +/- RF, ANA
```
Elevated ESR/CRP
Normocytic anemia
Thrombocytosis
Leukocytosis
Increased BUN/Cr
UA: proteinuria, RBC cast
```
66
granulomatosis with polyangiitis treatment
Cyclophosphamide: immunosuppressant which improves prognosis
Toxicities: CHF, thrombocytopenia, acute hemorrhagic cystitis, acute tubular necrosis, N/V/D
Corticosteroids
67
Hypersensitivity Pneumonitis
Repetitive inhalation of antigens in a susceptible host → inflammation
Antigens: bacteria, fungi, mold, proteins, chemicals, environments: bird/bat droppings, animal fur/feathers, hot tubs, hardwood dusts
4th-6th decade
68
Hypersensitivity Pneumonitis acute disease
remits without treatment
69
subtypes of Hypersensitivity Pneumonitis
Acute: flu-like syndrome within hours of exposure
Remits without therapy
Sub-acute: insidious onset of cough, dyspnea, fatigue over weeks
Chronic: progressive dyspnea, cough, fatigue, malaise
70
Hypersensitivity Pneumonitis symptoms
Diffuse, fine bibasilar crackles, fever, tachypnea, muscle wasting, clubbing, weight loss
71
Hypersensitivity Pneumonitis imaging
Interstitial inflammation
Honeycombing
Centrilobular fibrosis
Peribronchiolar fibrosis
72
Hypersensitivity Pneumonitis tx
Avoid antigen
Corticosteroids: speed recovery, chronic
adjunctive therapy: Bronchodilators, antihistamines, inhaled corticosteroids
73
Pulmonary hypertension/Cor pulmonale/CV disease
complication of interstitial lung disease
Lungs stiffen→ pulmonary arteries become dilated→ right ventricular dysfunction and enlargement
74
complications of interstitial lung disease
```
Pulmonary hypertension/Cor pulmonale/CV disease
Pneumothorax
PE
Pulmonary infection
Elevated cancer risk
Progressive respiratory insufficiency
```
75
Granulomatosis with polyangitis imaging
CXR: variable, nodules which may cavitate
CT chest:
→ stellate shaped peripheral pulmonary arteries= “vasculitis sign”
→ Feeding vessels to nodules/cavities
→ Diffuse alveolar hemorrhage
Tissue biopsy: histopathologic evidence of vasculitis, granulomatous inflammation
Labs:
