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X Clin Med II Exam 2 > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

Interstitial Lung Disease

A

Group of pulmonary disorders that cause progressive scarring of lung tissue surrounding the alveoli
Generally irreversible
Process of fibrosis and inflammation (not infectious)

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2
Q

Causes of interstitial lung disease

A

Idiopathic, autoimmune (dermatomyositis, RA), exposure to hazardous material or drugs (nitrofurantoin, methotrexate)

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3
Q

Sxs of interstitial lung disease

A

Progressive dyspnea on exertion
Persistent non-productive cough
*wheezing and chest pain (uncommon)
Extra pulm sxs (may suggestive connective tissue disease-MSK pain, weakness, joint pain, fevers, dry eyes)

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4
Q

What is seen on the physical exam in interstitial lung disease?

A

Crackles (usually at bases): velcro
Inspiratory squeaks (high pitched rhonchi)
Cor pulmonale: later stages
Cyanosis (only advanced disease)
Digital clubbing (advanced)
Extra pulm: associated with different disease like erythema nodusum of Gottrons papules)

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5
Q

Gold standard for diagnosis of ILD

A

Tissue biopsy (HRCT gives highest yield as non-invasive test)

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6
Q

What abnormal CXRs suggest ILD?

A

(10% have normal ones)
Ground glass appearance (early usually)
Reticular “netlike” (most common), nodular or mixed opacities
Honeycombing

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7
Q

What does honeycombing indicate?

A

Poor prognosis

Alveoli breakdown and become cystic and aren’t functional

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8
Q

What is HRCT used for?

A

Greater diagnostic accuracy than CXR
See distribution and pattern of disease in lung
Compare to old CT

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9
Q

Serologic studies in ILD

A

Rule out subclinical autoimmune disease (start with ANA and Rf and maybe more like anti-ds-DNA)
If hemorrhage or systemic sxs, evaluate for vasculitis (ANCA)
Sed rate and CRP not helpful

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10
Q

PFTs of ILD

A

Most are associated with restrictive defect (but might not see early on)
Decreased TLC
Decreased FEV1 and FVC but proportional so FEV1/FVC normal or increased

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11
Q

What should you do if the PFTs show a restrictive or normal pattern?

A

Obtain a DLCO (if low then consistent with ILD)

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12
Q

What is DLCO?

A

Diffusing Capacity of Lungs for Carbon monoxide (reduced might be the only finding in early disease)
Measures quantity of CO transferred each minute from alveoli to RBCs in pulm caps
Normal is 80% predicted

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13
Q

What does low DLCO signify?

A

Alveolar damage, impaired gas exchange and therefore less delivery of O2 into bloodstream

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14
Q

What might be seen on ABG in ILD?

A

Might be normal or show hypoxemia or respiratory alkalosis

May beed exercise testing with serial ABGs

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15
Q

What is bronchoalveolar lavage?

A

Minor extension of bronchoscopy
Allows sampling from distal airways and alveoli
Cell counts, cultures and cytology
Not typically performed with HRCT findings consistent with IPF

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16
Q

Indications for a lung biopsy

A

Specifying diagnosis (<50, weight loss, hemoptysis, signs of vasculitis)
Atypical or rapidly progressing HRCT findings or progressive disease
Unexplained extrapulm manifestation
Excluding neoplasm or infection that can mimic ILD
ID more treatable process than originally suspected
Predict likelihood of response to therapy

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17
Q

Contraindication for lung biopsy

A

Honeycombing (b/c poor prognosis so biopsy won’t change the management)

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18
Q

Transbronchial biopsy

A

During bronchoscopy, biopsy forceps passes through bronchoscope (helpful for central locations and not periphery)

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19
Q

Surgical biopsy

A

Done under anesthesia with 1 lung ventilated (larger sample)
Video assisted thorascopic surgery
Thoracotomy (5-6 cm incision to more pain after)

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20
Q

Endobronchial u/s-guided transbronchial needle aspiration

A

Special bronchoscope used to evaluate and mediastinal lymph nodes
Anesthesia
Especially useful if sarcoid suspected

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21
Q

Epidimiology of Idiopathic pulmonary fibrosis

A 
(50-85)
Males > females
Most common
If familial then present earlier
Poor prognosis (2-5 yr survival)
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22
Q

Presentation of idiopathic pulmonary fibrosis

A

Gradual onset of exertional dyspnea and non-productive cough (>6 mos)
Velcro crackle (inspiratory)
Digital clubbing

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23
Q

Diagnostics seen in IPF (CXR, HRCT, PFTs, echo)

A 
6 minute walk test for baseline
CXR: peripheral reticular opacities in bases, honeycombing
HRCT sees the same
PFTS: restrictive pattern
Don't need biopsy, bronchoscopy/BAL
Pulmonary HTN seen on echo
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24
Q

HRCT is used in IPF to determine if…

A

UIP pattern
Probable UIP pattern
Indeterminate UIP pattern
Alternative diagnosis

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25
What is a surgical lung biopsy + BAL used for in IPF
If there is not a definitive UIP pattern
26
Tx for IPF
``` Consult pulmonologist (not many choices tho) Treat for GERD even if no sxs Nintedanib (TKI) Pirfenidone Quit smoking and vaccines Supplement O2 prn ```
27
Use of pirfenidone in IFP
Reduces lung fibrosis through downregulation of production of growth factors and procollagens
28
Who is Ivan IPF?
Male 60ish Common man with not much PMH Smoker
29
Where does sarcoidosis mainly occur?
Lungs and intrathoracic lymph nodes (central predominance)
30
What do the non-caseating granulomas in sarcoidosis secrete?
``` 1/25 dihydroxyvitamin D so high serum levels (and Ca elevated too) Secrete ACE (acts as cytokine) ```
31
Epidimiology of sarcoidosis
Seen more in African Americans Slight female predominance 20-40
32
Presentation of sarcoidosis
DOE, chest pain, cough, hemoptysis is rare | Systemic complaints in about half
33
Extra pulmonary findings in sarcoidosis
Erythema nodosum, lupus pernios, granulomatous uveitis, arthralgias
34
What is seen on the diagnostics for sarcoidosis?
Restrictive patterns on PFTs (but 20% can be obstructive) May only see isolated reduced DLCO Adenopathy on CXR
35
Lab testing for sarcoidosis
Elevated serum ACE in 60% Elevated Ca, hypercalciuria Elevated alkaline phosphatase
36
Is a biopsy needed for sarcoidosis?
Usually (EBUS-TBLB is fine)
37
Staging for sarcoidosis
Based on CXR 0: normal 1: hilar adenopathy 2: hilar adenopathy and diffuse infiltrates 3: only diffuse parenchymal infiltrates 4: pulmonary fibrosis
38
When do pts usually start needing tx for sarcoidosis?
When stage 2 and symptomatic
39
Tx for sarcoidosis
High dose corticosteroids (taper over 6 mos-PJP prophylaxis) Methotrexate is non steroid alternative Chroloquine/hydroxychloroquine for cutaneous lesions, neurologic, hypercalcemia Lung transplant stage 4
40
Most common pattern of LAD in sarcoidosis
Bilateral symmetrical hilar and right parenchymal mediastinal adenopathy
41
Who is Sally Sarcoidosis?
``` Female African American 30s Non smoker (she has hilar adenopathy and shes an ACE) ```
42
What is pneumoconiosis?
General term for lung disease caused by inhalation and deposition of mineral dust Asbestosis, silicosis and pneumoconiosis
43
What characterizes silicosis?
Fibronodular lung disease | Due to inhalation of silica dust (alpha quartz or silicon dioxide)
44
Occupations at risk for silicosis
Mining, construction, granite cutting, pottery making
45
CXR for sarcoidosis
Enlarging opacities even after exposure eliminated (can cavitate so rule out TB)
46
PFTs for silicosis
Mostly restrictive pattern | Massive fibrosis leading to severe restriction
47
Chronic simple silicosis
10-12 yrs exposure (may be asymptomatic) Non-progressive when exposure is eliminated Hilar node calcification (eggshell pattern) Small round opacities (silicotic nodules) on CXR
48
Chronic complicated silicosis
>20 yrs exposure Progressive even after eliminate exposure Exam: tachypnea, prolonged expiration, rhonchi, wheezing,cyanosis and cor pulmonale (advance) Digital clubbing uncommon
49
What might benefit acute phase of silicosis?
Corticosteroids
50
Tx for silicosis
Consult pulmonologist Smoking cessation No specific therapy to alter disease course Vaccinate, treat latent TB because increased risk Maybe lung transplant if candidate
51
Who is Sam silicosis?
``` Male Miner Indeterminate age Smoker making things worse (has eggshell calcifications) ```
52
Epidimiology of asbestosis
Usually presents after 15-20 yrs exposure (dose dependent) 40-75 usually Males more
53
Occupations at risk for asbestosis
Construction (old building demo), auto mechanics, pipe fitters, plumbers, welders, janitors, shipyard, biolermakers
54
Asbestos and lung cancer
Asbestors is linked to lunger cancer and malignant mesothelioma Smoking increases the risk
55
Physical exam of asbestosis
``` No specific sxs Insidious onset of dyspnea, reduced exercise tolerance Dry cough End-inspiratory rales Digital clubbing (30-40%) ```
56
CXR asbestosis
Opacities in lower lungs, thickened pleura and calcified pleural plaques (reliable indicator)--usually in diaphragmatic pleura or parietal pleura of ribs 6-9
57
Open lung biopsy for asbestosis
Usually not indicated but would be definitive (histology shows fibrosis and asbestos bodies in light microscope)
58
PFTs asbestosis
Restrictive pattern (use to follow progression)
59
Tx of asbestosis
Consult pulm (long term O2) Immunotherapy drugs and steroids not effective Want to eliminate progression or sxs and reduce risk of lung disorders Vaccine for influenza and pneumovax
60
What is mesothelioma?
Form of cancer almost always associated with asbestos exposure Mesothelium is protective lining that covers most organs
61
Characteristics of mesothelioma
May be short term exposure (1-2 yrs) Most common in pleura but can be peritoneum or pericardium Not caused by smoking 6-12 month survival after presentation
62
Who is Al asbestosis?
``` Male 50ish Pipefitter Smoker making things worse (has pleural plaques) ```
63
What is granulomatosis with polyangiitis (GPA)?
Auto-immune mediated systemic vasculitis of small-medium vessels ANCA associated vasculitis Multisystem
64
What characterizes GPA?
Necrotizing granulomas of upper and lower respiratory tracts
65
Epidimiology of GPA
Northern European descent Men and women 35-55 Relapse common by 5 yrs
66
History in GPA
Recurrent respiratory infections | Nonspecific constitutional sxs (B sxs-fever, weight loss and night sweats, loss of appetite, fatigue)
67
Organ manifestations of GPA
``` Ocular involvement ENT (saddle nose! sinusitis, rhinitis) Pulmonary (infiltrates and cough common, dyspnea etc) MSK Renal Nervous system peripheral Skin (about half of people)- blistering purpura Cardiac ```
68
Diagnostic images in GPA
CXR: variable but maybe nodules that cavitate CT chest: stellate shaped peripheral pulmonary arteries (sign of vasculitis), feeding vessels leading to nodules and cavities, alveolar hemorrhage
69
Bronchoscopy in GPA
Usually not indicated unless hemorrhage or need for therapeutic intervention
70
Lab studies in GPA
ESR/CRP usually elevated CBC/CMP: normocytic anemia, thrombocytosis, leukocytosis, renal involvement UA: proteinuria if renal, RBC casts C-ANCA (antineutrophil cytoplasmic antibody) Maybe RF and ANA
71
Initial tx of GPA
Cyclophosphamide (immunosuppressant) and corticosteroids Watch for toxicites: cardiac, heme, renal, GI Consult rheum and pulm
72
What is hypersensitivity pneumonitis?
Inflammatory syndrome of lung caused by repetitive inhalation of antigens in susceptible host No predilections Bacteria/fungi, proteins, chems, environmental stuffs
73
Difference between hypersensitivity pneumonitis and the rest
This is reversible!! (but recovery make take a couple yrs)
74
History of hypersensitivity pneumonitis
flu-like syndrome within hours of exposure if acute Insidious onset of productive cough, dyspnea, fatigue over wks if subacute Progressive dyspnea, cough, fatigue if chronic
75
Physical exam of hypersensitivity pneumonitis
Diffuse, fine bibasilar crackles, fever, tachypnea, muscle wasting, clubbing
76
What is seen on imaging in hypersensitivity pneumonitis?
Interstitial inflammation, honeycombing, centrilobular fibrosis, peribronchiolar fibrosis
77
Tx for hypersensitivity pneumonitis
Consult pulm Avoidance Acute doesn't need therapy Corticosteroids can speed recovery when severe (so used in chronic) Bronchodilators, antihistamines, inhaled corticosteroids can be adjuncts
78
Complications of ILD
``` Pul HTN Cor pulmonale/cardiovascular disease (hypertrophy or dilation of right ventricle associated with lung disease) Pneumothorax PE Pulm infection Elevated cancer risk Progressive respiratory insufficiency ```

X Clin Med II Exam 2 (6 decks)

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