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Pulmonary Review > Pulmonary Vascular Diseases > Flashcards

Pulmonary Vascular Diseases Flashcards

1
Q

What are the small-vessel vasculitis?

A

ANCA associated (GPA, MPA, EGPA)

Immune complex associated (cryoglobulinemic, hypocomplementemic urticarial, Goodpastures, IgA)

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2
Q

What are the medium-vessel vasculitis?

A

PAN

Kawasaki disease

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3
Q

What are the large-vessel vasculitis?

A

Takayasu

Giant cell arteritis

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4
Q

45 y/o white male presents with sinusitis, hemoptysis, uveitis, and hematuria. Renal biopsy reveals crescentric segmental necrosis with pauci-immune immunofluorescence. Antiproteinase ANCA positive. Diagnosis?

A

GPA

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5
Q

38 y/o female from Italy presents with hypoxemia and is intubated. Bronchoscopy reveals DAH. She has worsening renal failure and biopsy reveals crescentric segmental necrosis. Serum is MPO-ANCA+. Diagnosis?

A

MPA

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6
Q

28 y/o female presents with worsening neuropathies and asthma symptoms. CT biopsy of lung nodules reveals necrotizing eosinophilic granulomatosis. Serum is MPO-ANCA+. Diagnosis?

A

EGPA

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7
Q

What medications are known to “unmask” EGPA when started?

A

Leukotriene inhibitors

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8
Q

Mainstay of medical treatment in all ANCA vasculitis in standard therapy with limited disiease (2 meds)?

A

Steroids and methotrexate

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9
Q

If an ANCA vasculitis has severe disease, you drop the methotrexate and add on what medications for each ANCA vasculitis?

A

GPA - steroids + cyclophosphamide (or rituxan)

MPA- steroids + rituxan

EGPA steroids + cyclophosphamide or rituxan or anti-IL-5

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10
Q

Mainstay of medical treatment in all ANCA vasculitis in remission in limited disease (2 meds)?

A

MTX or AZA

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11
Q

Mainstay of medical treatment in all ANCA vasculitis in remission in refractory disease?

A

Some combo of steroids + rituxan/cyclophosphamide (except no CYC in MPA) and plasma exchange

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12
Q

How often are pulmonary features seen in giant cell arteritis?

A

25%

lung nodules, interstitial infiltrates, bronchocentric granulomas

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13
Q

How often is pulmonary HTN seen in Takayasu arteritis?

A

50%

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14
Q

What infections is PAN associated with?

A

HBV

HCV

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15
Q

True/False: PAN is often associated with DAH as one of its complications

A

FALSE

This never causes capillaritis, so consider another cause.

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16
Q

Since lungs are affected in 50% of the population in anti-GBM disease, what is a common presenting symptom?

A

DAH

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17
Q

Although cryoglobulinemic vasculitis associated DAH is only seen in ~3% of patients, what is its mortality rate?

A

80%

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18
Q

Treatment of cryoglobulinemic vasculitis?

A

Rituxan

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19
Q

What % of patients with DAH present with hemoptysis?

A

30%

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20
Q

Tell me the 2 things that causes DAH with pulmonary capillaritis?

A

AAVs (GPA, MPA, EGPA)

Connective tiissue disorders (SLE, RA, mixed connective tissue disorders, Behcet disease, polymyositis, IgA-associated vasculitis)

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21
Q

Tell me the 4 things that causes DAH with or without pulmonary capillaritis?

A

Goodpastures
SLE
Antiphospholipid syndrome
Drug-induced DAH

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22
Q

Tell me the other things that causes DAH withOUT pulmonary capillaritis?

A 
Malignancies
LAM
PVOD
Pulmonary capillary hemangiomatosis
Mitral stenosis
Inhalation toxins (crack, trimetallic anhydride)
Idiopathic pulmonary hemosiderosis
Drugs (anticoagulation, abciximab, ATRA, sirolimus)
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23
Q

Is DAH more commonly
found in autologous or
allogenic BMT?

A

Autologous BMT

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24
Q

Treatment of DAH?

A

High dose steroids (500mg-2g/day) for 5 days followed by gradual tapering and maintenance

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25
Q

Since DAH associated with antiphospholipid syndrome has rapidly progressive respiratory failure and a high mortality rate, what therapy might be indicated in the treatment in addition to steroids?

A

Early plasma exchange

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26
Q

Case: 15 y/o presents with recurrent hemoptysis and GGO’s on imaging. Labwork reveals iron-deficiency. Bronchoscopy is performed and biopsy shows bland-lung histopathology with fibrosis. Diagnosis?

A

Idiopathic pulmonary hemosiderosis (IPH)

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27
Q

Treatment of IPH?

A

Immunosupression, plasma exchange, and gluten-free diet.

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28
Q

What is Lane-Hamilton

syndrome?

A 
Association of gluten-sensitive
sprue (celiac
sprue) with idiopathic
pulmonary hemosiderosis.
Only 18 cases are
described in the literature.
All IPH patients should be
screened for sprue with
serologies (antigliadin and
antiendomysial antibodies).
Gluten-free diet seems key
to therapy and prevention
of relapses.
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29
Q

Sensitivity/specificity of the following testing methods for PE?

D-Dimer
V/Q scan
Spiral CTA
Extremity US

A

D-Dimer 96/40

V/Q scan 77/98

Spiral CTA 83/96

Extremity US 29/97

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30
Q

Case: 25 y/o woman who is 34 weeks pregnant presents with dyspnea. No LE swelling or pain. You’re concerned for PE. CXR ordered and shows right lung opacity. Whats the next test?

A

CTPE

Abnormal CXR means you can’t do a V/Q, and high pre-test probability means you should proceed with the CT PE

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31
Q

Tell me the following anticoagulant based on the following mechanism of action:

Binds ATIII and factor Xa

A

Heparin

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32
Q

Tell me the following anticoagulant based on the following mechanism of action:

Binds factor Xa&raquo_space;» ATIII

A

Enoxaparin

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33
Q

Tell me the following anticoagulant based on the following mechanism of action:

Blocks vitakin K epoxide, slow decrease in factors II, VII, IX, X, and thrombin

A

Warfarin

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34
Q

Tell me the following anticoagulant based on the following mechanism of action:

Direct thrombin inhibitor

A

Argatroban

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35
Q

Tell me the following anticoagulant based on the following mechanism of action:

Oral factor IIa inhibitor

A

Dabigatran

36
Q

Tell me the following anticoagulant based on the following mechanism of action:

Oral factor Xa inhibitor

A

Rivaroxaban (or the others of course)

37
Q

Tell me the following anticoagulant based on the following mechanism of action:

Increased plasminogen, which increases plasmin and therefore breaks down fibrin

A

Thrombolytic therapy (streptokinase, urokinase, tPA)

38
Q

Mortality rates for the following scenarios in PEs?

RV dysfunction, no hypotension

Hypotension

Cardiogenic shock

Cardiopulmonary resuscitation

A

RV dysfunction, no hypotension - 8%

Hypotension - 15%

Cardiogenic shock - 25%

CPR - 65%

39
Q

Fill in the blank for “potential” indications for tPA in PE:

Severe \_\_\_\_
Large perfusion defect on \_\_ scan
Extensive \_\_\_ \_\_\_ on CT
\_\_ dysfunction
Free-floating RA or ventricular \_\_\_
PFO
CPR
A 
Severe HYPOXEMIA
Large perfusion defect on CT scan
Extensive EMBOLIC BURDEN on CT
RV dysfunction
Free-floating RA or ventricular THROMBUS
PFO
CPR
40
Q

Fill in the blank for indications for tPA in PE:

Persistent ___
Shock
Massive proximal LE ___ associated with severe swelling or limb ischemia

A

Persistent HYPOTENSION

Shock

Massive proximal LE THROMBUS associated with severe swelling or limb ischemia

41
Q

Fill in the blank for relative contraindications for tPA in PE:

Severe uncontrolled HTN (BP >___)
Hx of prior ___ not covered in absolute contraindications
Current use of ___; known bleeding diathesis
Recent trauma (within 2-4 weeks), prolonged CPR (>10 mins) or major ___ within 3 weeks
Noncompressible vascular punctures
Recent (2-4 wks) __ bleeding
Pregnancy, PUD

A

Severe uncontrolled HTN (BP >180/110)

Hx of prior CVA not covered in absolute contraindications

Current use of ANTICOAGULANTS; known bleeding diathesis

Recent trauma (within 2-4 weeks), prolonged CPR (>10 mins) or major SURGERY within 3 weeks

Noncompressible vascular punctures

Recent (2-4 wks) INTERNAL bleeding

Pregnancy, PUD

42
Q

Fill in the blank for ABSOLUTE contraindications for tPA in PE:

Previous ___ stroke at ___; other stroke or cerebrovascular events within 1 year
Known intracranial ___
Active ___ bleeding
Suspected ___ dissection

A

Previous HEMORRHAGIC stroke at ANY TIME; other stroke or cerebrovascular events within 1 year

Known intracranial NEOPLASM

Active INTERNAL bleeding

Suspected AORTIC dissection

43
Q

How is pulmonary vascular
resistance calculated in
Wood units?

A

(mPAP – PCWP) ÷ cardiac
output (L/min) = PVR in
Wood units

44
Q

What is the most common
cause of pulmonary
hypertension?

A 
Left-sided systolic or
diastolic heart disease
(WHO Group 2), although
this represents pulmonary
venous hypertension rather
than pulmonary arterial
hypertension.
45
Q

What are common
drugs/toxins implicated in
pulmonary hypertension?

A

Aminorex, fenfluramine,
dexfenfluramine,
amphetamines,
methamphetamines

46
Q

Mutations in what gene are detected in ~70% of PAH cases and 11-40% of IPAH patients?

A

BMPR2

Also see ALK-1 mutations

47
Q

What are the 3 main pathways that cause PAH?

think medication targeted pathways

A 
Endothelin pathway (endothelin-1)
NO pathway
Prostacyclin pathway (PI2)
48
Q

Imbalance of vasoconstriction + proliferation and vasodilation + anti-proliferation is what causes PAH. Which pathways are vasoconstrictive, and which are vasodilatory?

A

Vasocontrictive/proliferative = endothelin pathway

Vasodilatory/anti-proliferative = NO, PI2

49
Q

Tell me the 4 WHO functional classifications for PAH.

A

Class I - no limitation on activity

Class II - slight limitation on activity, usually some symptoms with activity

Class III - marked limitation on activity, comfortable at test, symptoms with any activity

Class IV - symptoms at rest, unable to perform activities

50
Q

How is a positive
vasoreactivity test defined
during RHC?

A 
A positive response to
therapy with vasodilators is
defined as a fall in mean
PAP of > 10 mm Hg to
reach a mean PAP of < 40
mm Hg with an increased
or unchanged cardiac
output.
51
Q

How does one establish a
definitive diagnosis of
PAH?

A 
With RHC showing a
mPAP ≥ 25 mm Hg with
pulmonary capillary wedge
pressure ≤ 15 mm Hg and
pulmonary vascular
resistance > 3 Wood units
(240 dynes/sec/cm5).
52
Q

What medciations can be trialed if patient has a positive vasoreactivity testing?

A

CCBs

53
Q

Which group of drugs is
associated with
hepatotoxicity?

A

Endothelin receptor

antagonists (ERAs)

54
Q

Which group of drugs is
contraindicated with the
use of nitrates?

A

Phosphodiestrase-5 (PDE-

5) inhibitors

55
Q

Which group of drugs

causes teratogenicity?

A

Endothelin receptor

antagonists (ERAs)

56
Q

Tell me the 3 prostanoids

A

Epoprostenol (IV)
Iloprost (inhaled)
Treprostinil (IV, SC, inhaled)

57
Q

Tell me the 3 ERAs

A

Bosentan
Ambrisentan
Macitentan

58
Q

Tell me 2 PDE-5 inhibitors

A

Sildenafil

Tadalafil

59
Q

Tell me the 1 soluble guanylate cyclase stimulator

A

Riociguat

60
Q

Based on the following adverse effects, which PROSTANOID am I talking about?

Flushing, headache, nausea, vomiting, thrombocytopenia, hypotension, jaw pain

A

Epoprostenol

61
Q

Based on the following adverse effects, which PROSTANOID am I talking about?

Flushing, cough, headache, nausea, flu-like illness

A

Iloprost

62
Q

Based on the following adverse effects, which PROSTANOID am I talking about?

Site pain and erythema in SC administration, headache, nausea, diarrhea, cough(inhaled)

A

Treprostinil

63
Q

Based on the following adverse effects, which ERA am I talking about?

Hepatotoxicity (stop if bilitubin 2x ULN, AST/ALT 3x ULN), edema, nasopharyngitis, teratogenic

A

Bosentan

64
Q

Based on the following adverse effects, which ERA am I talking about?

AST/ALT elevation (stop if >8x ULN), edema, headache, nasal congestion, flusing, teratogenic

A

Ambrisentan

65
Q

Based on the following adverse effects, which ERA am I talking about?

URI, edema, nasopharyngitis, headache, anemia, AST/ALT elevation, teratogenic

A

Macitentan

66
Q

Based on the following adverse effects, which PDE-5 inhibitor am I talking about?

Headache, dyspepsia, flusing, epistaxis, optic neuritis (rare), potentiates hypotension with other meds

A

Sildenafil

67
Q

Based on the following adverse effects, which PDE-5 inhibitor am I talking about?

Headache, flushing, respiratory infections, extremity pain, avoid if renal or hepatic impairment

A

Tadalafil

68
Q

What are the side effects of Riociguat?

A

Headache, dyspepsia, nausea, peripheral edema

69
Q 
Which of these categories
of PAH has the best
prognosis?
A. PAH associated with
congenital heart
disease
B. PAH associated with
scleroderma
C. PAH associated with
HIV
D. PAH associated with
liver disease
A

A. PAH associated with

congenital heart disease.

70
Q

PASP > what value is suggestive of CTEPH?

A

> 50mmHg

71
Q

What test is the gold standard to diagnose CTEPH?

A

VQ scan

72
Q

Treatment of CTEPH?

A

Pulmonary endarterectomy
Riociguat (in those who are not surgical candidates)
Anticoagulation (duhhh)

73
Q

This type of pulmonary HTN is where there is fibrotic remodeling of the intima of preseptal pulmonary venules and interlobular septal veins which causes flow obstruction, often with intaluminal fibrosis.

A

PVOD

74
Q

What gene plays a role in
the pathogenesis of
PVOD?

A

BMPR-2

75
Q

True/False: PVOD can also affect pulmonary arterioles resulting in similar remodeling as IPAH.

A

TRUE

The only difference is that PVOD usually lacks arteriolar plexiform lesions

76
Q

So since PVOD can look like PAH on cath and what not, what changes on the following items supports PVOD as the diagnosis?

Chest imaging
BAL results
Response to PAH therapy

A

Imaging has pulmonary edema with a normal PCWP

BAL shows alveolar hemorrhage

PAH-specific therapy causes the development of pulmonary edema

77
Q

Treatment of PVOD?

A

Still use PAH-specific treatments but you have to be careful as they usually get pulmonary edema, so keep them in the ICU while you diurese them

78
Q

Prognosis of PVOD?

A

1 year mortality is 72%, so early transplant referral

79
Q

What is seen on imaging for acute chest syndrome in sickle cell disease?

A

Usually consolidation involving 1 complete lung segment

80
Q

Treatment of acute chest syndrome?

A

Supportive:

Analgesics
Hydration
Supplemental O2
Blood transfusion
ISB
Antibiotics
SABAs for bronchospasm
Exchange transfusion for severe illness
81
Q

What is the most common
pathogen identified in
patients with acute chest
syndrome?

A

Chlamydia pneumoniae

82
Q

Treatment of fat emboli syndrome?

A

Supportive

Exchange transfusion if severe

83
Q 
What molecule is felt to be
primarily responsible for
the intrapulmonary
vascular dilations seen in
HPS?
A

NO

84
Q

What is the most
commonly used method for
confirming IPVD in HPS
patients?

A

Contrast enhanced

transthoracic echo

85
Q

What intervention has been
shown to improve
oxygenation and survival in
HPS patients?

A

Liver transplantation

86
Q

What herb has been shown to have some improvement in PaO2 in HPS?

A

Garlic

Pulmonary Review (14 decks)

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