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Pulmonary Boards > Vascular Disease > Flashcards

Vascular Disease Flashcards

1
Q

What are the features of Goodpasture syndrome?

A

Histo: capillaritis on renal biopsy with crescent formation, hemosiderin-laden macrophages
Features: pulmonary-renal syndrome that can cause DAH and RPGN, rare and in young adults
Imaging: GGO and diffuse alveolar infiltrates
Dx: anti-GBM antibodies
Tx: steroids, cyclophosphamide, plasmaphoresis

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2
Q

What complication can arise from pulmonary endarterectomy?

A

Reperfusion pulmonary edema (can see on repeat VQ scan) within 72hr in 30% of patients

Risk factors include preoperative RAP>12 and TPR over 1000 dynes

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3
Q

What factors are associated with higher mortality in patients with sickle cell disease associated PAH?

A

TRV above 2.5 and BNP above 160, screening echos recommended

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4
Q

What are the features of granulomatosis with polyangiitis?

A

Histo: granulomas and vasculitis
Features: weight loss, cough, hemoptysis, cystic lesions, upper airway issues like sinusitis
Imaging: cysts, can evolve to DAH
Dx: ANCA positive (but can be seronegative)
Tx: steroids and cyclophosphamide to induce remission (plasmaphoresis sometimes in severe organ-threatening disease), maintenance therapy with ritux, azathioprine, MTX, or sometimes MMF (less effective)

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5
Q

How do HIT types I and II differ?

A

Type I- onset 1-4 days, nadir of about 100k, non-antibody mediated, no hemorrhagic sequelae, and manage with observation

Type II- onset 5-10 days, nadir 20-80k, antibody mediated, can have rare hemorrhagic sequelae, and must change anticoagulant

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6
Q

How are PDE5i tolerated in patients with PAH due to sickle cell disease?

A

Try to avoid, have increased hospitalizations due to pain crisis

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7
Q

Which patients with newly diagnosed DVT can be managed outpatient?

A

Stable, ambulatory with VSS, no increased risk of bleeding, normal renal function, a practical system in place for follow up and monitoring, no concurrent PE, phlegmasia cerulea dolens, or other comorbidities that require inpatient care

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8
Q

How are hepatic AVMs causing high output heart failure treated in HHT?

A

Liver transplantation. While we embolize pulmonary AVMs, liver embolization has a high potential for potentially fatal hepatic necrosis. Bevacizumab (VEGF antibody) can transiently help, but not usually a permanent solution.

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9
Q

How long should anticoagulation be continued in pregnant patients with DVT/PE?

A

Throughout pregnancy and then 6 weeks post-partum to complete a total of 3 months. LMWH and UFH can be used, but LMWH is preferred due to less complications with osteoporosis. Hold 24hrs prior to induction, high risk patients can transition to UFH 24 hrs prior and hold 6-12hrs prior to delivery

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10
Q

What risk factors are associated with pulmonary artery pseudoaneurysms?

A

Infection (septic emboli, TB/Rasmussen, syphilis, pyogenic bacteria, fungi), complications (from PA catheters, chest tube insertion, biopsies, penetrating chest trauma), malignancies (bronchogenic SCC, primary sarcoma, metastatic sarcoma), chronic inflammation (Behcet, takayasu arteritis)

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11
Q

How would one workup extramedullary hematopoiesis?

A

See a hypovascular soft tissue mass with areas of fat attenuation, unusual to have calcifications. Can consider MRI to better characterize as biopsy is high risk for bleeding due to increased vascularity.

Seen often in patients with myelofibrosis, metastatic skeletal disease, leukemia, SCD, thalassemia

Most often seen in liver/spleen, then paraspinal

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12
Q

Based off YEARS study for work up of PE in pregnant women, which criteria must be met to rule out PE before CTA?

A

Signs of DVT, hemoptysis, and PE being most likely with D-dimer below 1000 (or if only one criteria was met with D-dimer under 500). Otherwise can rule out with CTA.

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13
Q

How long to anticoagulate empirically a patient without bleeding risk who undergoes hip fracture surgery?

A

10-14days

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14
Q

What is the threshold for holding anticoagulation in patients with ITP?

A

Experts suggest 30k

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15
Q

What is a potential but deadly complication of treating HRS with terlipressin and albumin?

A

Respiratory failure

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16
Q

What is the reversal agent for different anticoagulants?

A

Dabigatran–> idarucizumab, four factor prothrombin complex concentrate
Andexanet alfa–> direct factor Xa inhibitors (apixaban/edoxaban/rivaroxaban)
Warfarin- four factor prothrombin complex concentrate
Cryoprecipitate- hypofibrinogenic states such as von Willebrand factor deficiency, factor XIII deficiency, or hemophilia

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17
Q

What are the features of pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis?

A

Histo- hemosiderin laden macrophages on BAL
Features: LE edema, DOE, PAH symptoms, hemoptysis
Imaging: interlobular septal thickening, ground glass mosaic attenuation
Dx: mutations in EIF2AK4, sometimes BMPR2
Tx: NOT vasodilator therapy (can worsen pulmonary edema)

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18
Q

How should DVT/PE ppx be managed in subsequent pregnancies?

A

LMWH/UFH in prophylactic dose, throughout the pregnancy and 6 weeks postpartum. Warfarin can’t be used in pregnancy but can be used post-partum. DOACs have not been studied well enough during pregnancy or with lactation. Fondaparinux for HIT patients.

19
Q

How does one with HHT screen for pulmonary AVMs?

A

Bubble echo (late entry of agitated saline). Then confirm with CTA chest. Embolize all pulmonary AVMs that can be reasonably embolized.

20
Q

What is the Curacao diagnostic criteria for HHT?

A

3 out of 4 criteria: recurrent and spontaneous epistaxis, telangiectasias on hands/face/inside nose/mouth, AVM/telangiectasias in an organ (lung, brain, liver, intestine, stomach, spinal cord), FHx in a first degree relative.

80% have ENG or ACVRL1 mutation

21
Q

Recall the different waveforms for RAP, RVP, PAP, and PCWP

A
22
Q

How are pre, post, and combined capillary PAH defined?

A

Pre- PCWP < 15, PVR > 3 (groups 1,3, 4)
Post- PCWP > 15 , PVR < 3 (group 2)
Combined- PCWP > 15, PVR > 3 (group 2, 5)

23
Q

What are the genetic mutations associated with heritable PAH?

A

BMPR2 (75% of HPAH and 25% of IPAH), ALK-1 (HHT mutation), EIF2AK4 (PVOD/PCH)

24
Q

When should those with CTD start annual screening for PAH?

A

SSc and SSc spectrum with DLCO <80

25
Q

What are the TPG and DPG calculations?

A

TPG= mPAP - mean PCWP (normal under 12)
DPG= diastolic PAP - mean PCWP (normal under 7)

26
Q

What is indicative of a positive vasodilator challenge in RHC?

A

Drop in mPAP >10 to a mean under 40, no decline in CO/CI, no rise in PCWP

Only indicated for IPAH/DT/HPAH patients. Repeat RHC in 3-6 months to eval for long term response- 1/2 lose reactivity over time

27
Q

What is the likelihood of PE if no PERC criteria is met?

A

Less than 2%, no further eval needed, including Ddimer

28
Q

What level of Ddimer is recommended to rule out PE?

A

Age adjusted- 10x patient’s age

29
Q

Which patient populations should not be tested with Ddimer to rule out PE?

A

Symptoms of VTE for 14+ days, suspected VTE on heparin, pregnant or receiving oral AC

30
Q

When can CTPE be considered in pregnant patients?

A

Patient with high suspicion and -US, nondiagnostic V/Qs, or abnormal CXRs

31
Q

Which DOACs need to have parenteral overlap for 5-10 days for initiation? (due to the initial trials)

A

Dabigatran and edoxaban

32
Q

What are the common large and medium vessel vasculitis?

A

Giant cell- most common vasculitis but rare pulm complications, can have cough and nodules

Takayasu- young females, PA aneurysm/stenosis/infarct, rare

Polyarteritis Nodosa- 3 NO’s: no ANCA, no pulm capillaritis, no GN, can have bronchial artery involvement

Behcet’s- can have PA aneurysm

33
Q

How can the small vessel vasculitis be classified?

A

ANCA- associated: Microscopic, GPA (formerly Wegner’s), EGPA (formerly Churg-Strauss)

Immune-complex associated: Anti-GBM (Goodpasture), IgA (Henoch-Schonlein), cryoglobulinemic, anti-C1q

34
Q

What are the differences between pANCA and cANCA diseases

A
35
Q

How is severe MPA/GPA treated?

A

RTX over CYC and reduced dose GC. If remission –> RTX vs MTZ or AZA or MMF or LEF
If no remission –> Switch to different induction agent versus combination

36
Q

How is non-severe GPA treated?

A

GC+MTX (preferred over GC+ RTX/CYC/AZA/MMF/monotherapy)

If remission–> continue MTX/AZA/MMF or switch

37
Q

In ANCA associated DAH, is PLEX useful?

A

According to PEXIVAS trial, no. Also then has increased risk of infection.

Only consider with double positivity with ANCA and anti-GBM, high risk to develop ESRD, or as salvage therapy

38
Q

Do studies suggest usefulness in C5a receptor inhibitor Avacopan in DAH?

A

Was superior to steroid taper in sustained remission but similar rates of short term remission

39
Q

How is severe EGPA treated?

A

High dose steroids + CYC vs RTX

If remission on CYC–> switch to MTX/AZA/MMF

Relapse –> High dose steroids + RTX

40
Q

How is non-severe EGPA treated?

A

GC + MEP vs MTX/AZA/MMF vs RTX vs monotherapy

If relapse on MTX/AZA/MMF/monotherapy –> GC + MEP

Relapse on MEP or RTX –> consider different approach

41
Q

How many hemosiderin-laden macrophages will you see in DAH?

A

> 20%, can take a few hours for them to form and can stay up to 6-8 weeks

42
Q

What clinical scenarios can cause bland hemorrhage/capillary stress failure?

A

Rupture of the capillaries is due to elevation of capillary transmural pressure (severe mitral valve stenosis)

43
Q

What are the best predictors in DAH for respiratory failure?

A

p/f ration <450, CRP >25, and BAL neutrophils >30%

44
Q

What are the features of idiopathic pulmonary hemosiderosis?

A

Diagnosis of exclusion
Features: recurrent hemoptysis and GGO, bland histopathology. mostly children, adults 20-40
Tx: GC and immunosuppressive agents
Associated with Lane-Hamilton syndrome (gluten-free diet)

Pulmonary Boards (14 decks)

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