Pulm: Vasculitides And Pulm Htn

Front 1

Pulm AVMs

Back 1

• Idiopathic or aquired but think HHT (hereditary hemorrhagic telangiectasia)
• Cause R to L shunting to causing brain abscess stroke or hypoxemia
• Think pulm AVM in a young person w/ a stroke

Hint 1

no hints. quit being lazy/dumb

Front 2

Pulmonary Vasculitides- a list

Back 2

• Granulomatosis w/ polyangiitis (Wegeners)
• Eosinophilic Granulomatosis w/ Polyangiitis (Churg-Strauss)
• Giant Cell Arteritis
• Behcet Disease
• Takayasu Arteritis
• Goodpasture syndrome
• Microscopic Polyangiitis

Front 3

Granulomatosis w/ polyangiitis (Wegeners)

Back 3

• SMALL Vessels, C-ANCA+, Proteinase 3+
• Necrotizing granulomas: EARS, NOSE, THROAT, LUNGs, KIDNEYs, SKIN
• Clinical Scenario: White patient w/ nasal septal perforation/ulceration of vomer bone (saddle nose deformity) w/ systemic disease and major resp manifestations w/ renal involvement
• Txt: Corticosteroids/cyclophosphamide, prevent relapse w/ bactrim?

Front 4

Saddlenose Deformity

Back 4

Differential:

Wegeners

Relapsing polychodritis

leprosy

Front 5

Eosinophilic Granulomatosis w/ Polyangiitis

(Churg-Strauss Syndrome)

Back 5

• Sx: REFRACTORY ASTHMA, Allergic rhinitis, nasal polyps, nasal mucosal crusting, and septal perforation
• Labs: Eosinophilia/Inc IgE levels
• Path: characterized by pulmonary and systemic vasculitis, extravascular granulomas

Front 6

Giant Cell Arteriitis

Back 6

• MEDIUM VESSEL
• Clinical Scenario: Older patient w/ new cough or throat pain, pulm nodules
• Histopath: mononuclear infiltrates w/ giant cells
• Txt: Corticosteroids

Front 7

Behcet Disease

Back 7

• Aphthous orogenital ulcerations, uveitis, cutaneous nodules, meningoencephalitis
• causes pulm vessel inflammation
• Vascular-Airway Fistulas common, 39% of patient die of severe hemoptysis
• high incidence of DVT/PE

Front 8

Takayasu Disease

Back 8

• MEDIUM/LARGE Vessels
• Pulm involvement is poor prognostic indicator
• pulmonary arteries are involved >50% of pts
• Txt: Cortcosteroids

Front 9

Goodpasture Syndrome

Back 9

• Cytotoxic Type II Rxn, P-ANCA, MPO+
• Anti-GBM +, (also + in flu virus, hydrocarbons, penicillamine, SLE etc)
• Alveolar Hemorrhage always precedes renal sx
• Clinical Scenarios: 1. Young man w/ GN and lung hemorrhage. 2. Elderly woman w/ GN alone.
• TXT: Plasmaparesis

Front 10

Microscopic Polyangiitis

Back 10

• DISTINCT FROM PAN
• Clinical Feature: PROGRESSIVE GN, Pulm hemorrhage in 12-29%
• P-ANCA+, MPO+

Front 11

Pulmonary HTN Types

Back 11

1. Idiopathic/Acquired from Infxn(HIV), Congenital Heart disease, drug associated
2. Systolic/Diastolic/Valvular Heart Disease
3. Lung Disease or Hypoxia (COPD, OSA, high altitude)
4. Chronic thromboembolic Pulm HTN
5. Systemic Disorders (HEME (myeloproliferative), Sarcoid, Thyroiditis, chronic renal failure)

Front 12

PAH

Back 12

• Part of Class I, defined as mean PAP >25, or wedge pressure >15
• Etiologies generally unknown
• Dx: Initial TTE then MUST GET RHC and assess vasodilator responsiveness to see if CCB are an option
• Txt: Below but eventually all patients need a lung transplant

Front 13

PAH treatment

Back 13

CCB: only for patients w/ acute vasodilator response during RHC

Prostanoids (epoprostenol, treprostineil,iloprost): supplements endogenous levels of prostacyclin which stimulates vasodilation and anti-smooth muscle proliferation

Endothelin-1 Receptor Antagonists(Bosentan, Ambrisentan): blocks vasoconstrictor activity of endothelin (teratogenic and liver injury)

PDE-5 inhibitors (sildenafil, tadalafil): prolongs effect of intrinsic vasodilator cyclic GMP by inhiniting hydrolysis of PDE-5

Front 14

PFTs

Obstructive

Back 14

• Obstructive FEV1/FCV ratio <70%,
• "Scooped out appearing"

Front 15

PFTs

Restrictive

Back 15

• Restrictive FEV1 and FCV both dec with normal FEV1/FCV ratio AND TLC <80% normal

Front 16

PFTs

Graphs/Special Cases

Back 16

• Tracheal Stenosis
• Getting a flow-volume loop is the dx tool.
• Px: Insp/Exp wheezing
• Always a young MVA patient.

Front 17

PFTs

Graphs/Special Cases

Back 17

• Interstitial lung disease: Dec TLC Dec Residual Volume
• Respiratory Muscle Weakness: Dec TLC and INCREASED residual volume (bc of inability to fully exhale)