Pulmonary Vascular Diseases Flashcards by Daniel Guck

What are the small-vessel vasculitis?

ANCA associated (GPA, MPA, EGPA)

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

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What are the medium-vessel vasculitis?

PAN

Kawasaki disease

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What are the large-vessel vasculitis?

Takayasu

Giant cell arteritis

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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?

GPA

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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?

MPA

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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?

EGPA

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

Leukotriene inhibitors

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

Steroids and methotrexate

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

GPA - steroids + cyclophosphamide (or rituxan)

MPA- steroids + rituxan

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

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

MTX or AZA

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

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

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How often are pulmonary features seen in giant cell arteritis?

25%

lung nodules, interstitial infiltrates, bronchocentric granulomas

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How often is pulmonary HTN seen in Takayasu arteritis?

50%

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What infections is PAN associated with?

HBV

HCV

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

FALSE

This never causes capillaritis, so consider another cause.

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

DAH

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

80%

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Treatment of cryoglobulinemic vasculitis?

Rituxan

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What % of patients with DAH present with hemoptysis?

30%

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

AAVs (GPA, MPA, EGPA)

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

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

Goodpastures
SLE
Antiphospholipid syndrome
Drug-induced DAH

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

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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Is DAH more commonly
found in autologous or
allogenic BMT?

Autologous BMT

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Treatment of DAH?

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

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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?

Early plasma exchange

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?

Idiopathic pulmonary hemosiderosis (IPH)

Treatment of IPH?

Immunosupression, plasma exchange, and gluten-free diet.

What is Lane-Hamilton | syndrome?

``` 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. ```

Sensitivity/specificity of the following testing methods for PE? D-Dimer V/Q scan Spiral CTA Extremity US

D-Dimer 96/40 V/Q scan 77/98 Spiral CTA 83/96 Extremity US 29/97

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?

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

Tell me the following anticoagulant based on the following mechanism of action: Binds ATIII and factor Xa

Heparin

Tell me the following anticoagulant based on the following mechanism of action: Binds factor Xa >>>> ATIII

Enoxaparin

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

Warfarin

Tell me the following anticoagulant based on the following mechanism of action: Direct thrombin inhibitor

Argatroban

Tell me the following anticoagulant based on the following mechanism of action: Oral factor IIa inhibitor

Dabigatran

Tell me the following anticoagulant based on the following mechanism of action: Oral factor Xa inhibitor

Rivaroxaban (or the others of course)

Tell me the following anticoagulant based on the following mechanism of action: Increased plasminogen, which increases plasmin and therefore breaks down fibrin

Thrombolytic therapy (streptokinase, urokinase, tPA)

Mortality rates for the following scenarios in PEs? RV dysfunction, no hypotension Hypotension Cardiogenic shock Cardiopulmonary resuscitation

RV dysfunction, no hypotension - 8% Hypotension - 15% Cardiogenic shock - 25% CPR - 65%

# 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 ```

``` Severe HYPOXEMIA Large perfusion defect on CT scan Extensive EMBOLIC BURDEN on CT RV dysfunction Free-floating RA or ventricular THROMBUS PFO CPR ```

# Fill in the blank for indications for tPA in PE: Persistent ___ Shock Massive proximal LE ___ associated with severe swelling or limb ischemia

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

# 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

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

# 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

Previous HEMORRHAGIC stroke at ANY TIME; other stroke or cerebrovascular events within 1 year Known intracranial NEOPLASM Active INTERNAL bleeding Suspected AORTIC dissection

How is pulmonary vascular resistance calculated in Wood units?

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

What is the most common cause of pulmonary hypertension?

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

What are common drugs/toxins implicated in pulmonary hypertension?

Aminorex, fenfluramine, dexfenfluramine, amphetamines, methamphetamines

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

BMPR2 Also see ALK-1 mutations

What are the 3 main pathways that cause PAH? | think medication targeted pathways

``` Endothelin pathway (endothelin-1) NO pathway Prostacyclin pathway (PI2) ```

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

Vasocontrictive/proliferative = endothelin pathway Vasodilatory/anti-proliferative = NO, PI2

Tell me the 4 WHO functional classifications for PAH.

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

How is a positive vasoreactivity test defined during RHC?

``` 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. ```

How does one establish a definitive diagnosis of PAH?

``` 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). ```

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

CCBs

Which group of drugs is associated with hepatotoxicity?

Endothelin receptor | antagonists (ERAs)

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

Phosphodiestrase-5 (PDE- | 5) inhibitors

Which group of drugs | causes teratogenicity?

Endothelin receptor | antagonists (ERAs)

Tell me the 3 prostanoids

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

Tell me the 3 ERAs

Bosentan Ambrisentan Macitentan

Tell me 2 PDE-5 inhibitors

Sildenafil | Tadalafil

Tell me the 1 soluble guanylate cyclase stimulator

Riociguat

Based on the following adverse effects, which PROSTANOID am I talking about? Flushing, headache, nausea, vomiting, thrombocytopenia, hypotension, jaw pain

Epoprostenol

Based on the following adverse effects, which PROSTANOID am I talking about? Flushing, cough, headache, nausea, flu-like illness

Iloprost

Based on the following adverse effects, which PROSTANOID am I talking about? Site pain and erythema in SC administration, headache, nausea, diarrhea, cough(inhaled)

Treprostinil

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

Bosentan

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

Ambrisentan

Based on the following adverse effects, which ERA am I talking about? URI, edema, nasopharyngitis, headache, anemia, AST/ALT elevation, teratogenic

Macitentan

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

Sildenafil

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

Tadalafil

What are the side effects of Riociguat?

Headache, dyspepsia, nausea, peripheral edema

``` 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. PAH associated with | congenital heart disease.

PASP > what value is suggestive of CTEPH?

>50mmHg

What test is the gold standard to diagnose CTEPH?

VQ scan

Treatment of CTEPH?

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

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.

PVOD

What gene plays a role in the pathogenesis of PVOD?

BMPR-2

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

TRUE The only difference is that PVOD usually lacks arteriolar plexiform lesions

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

Imaging has pulmonary edema with a normal PCWP BAL shows alveolar hemorrhage PAH-specific therapy causes the development of pulmonary edema

Treatment of PVOD?

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

Prognosis of PVOD?

1 year mortality is 72%, so early transplant referral

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

Usually consolidation involving 1 complete lung segment

Treatment of acute chest syndrome?

Supportive: ``` Analgesics Hydration Supplemental O2 Blood transfusion ISB Antibiotics SABAs for bronchospasm Exchange transfusion for severe illness ```

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

Chlamydia pneumoniae

Treatment of fat emboli syndrome?

Supportive Exchange transfusion if severe

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

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

Contrast enhanced | transthoracic echo

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

Liver transplantation

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

Garlic