Vasculitis Flashcards

(44 cards)

1
Q

What two types of Vasculitis affect Large Vessels?

A
  • Temporal (Giant Cell) Arteritis
  • Takayasu Arteritis
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2
Q

What are the 3 types of Vaculitis that affect medium vessels?

A
  • Polyarteritis Nodosa
  • Kawasaki Disease
  • Buerber Disease
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3
Q

What are the 6 diseases causing small vessel Vasculitis?

A
  • Wegener Granulomatosis
  • Microscopic Polyangiitis
  • Churg-Strauss Syndrome
  • Behcet Disease
  • Henoch-Schonlein Purpura
  • Crglobulinema
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4
Q

Temporal (Giant Cell) Arteritis

  • type of vasculitis
  • typical patient profile
  • symptoms/complications
  • important Lab Values
  • mediating Cell Type
  • Treatment
A

Large Vessel Vasculitis

Type: Granulomatous Vasculitis

Patient Profile: >50 White Females

Symptoms/complications: Headache, Jaw Claudication, Visual Changes, Blindness

Labs: Elevated ESR

Mediating Cell Type: T Cell Mediated

Treatment: steriods

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5
Q

Polymyalgia Rheumatica

  • Symptoms
  • Labs
  • Treatment
A

Symptoms: Proximal Muscle Aches and Pains

Labs: Elevated ESR (>100)

Treatment: Steroids = rapid resolution

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6
Q

Takayasu Arteritis

  • type of vasculitis
  • typical patient profile
  • symptoms/complications
  • important Lab Values
  • mediating Cell Type
  • Treatment
A

Large Vessel Vasculitis

Type: Graunlomatous Vasculitis

Patient Profile: Adults under 50 y/o (often Asian Females)

Symptoms/Complications: Visual and Neurologic symptoms with Weak or Absent Upper Extremity Pulse, CLASSICALLY INVOLVES AORTIC ARCH BRANCHPOINTS

Labs: Elevated ESR

mediating cell type: not specified

Treatment: steroids - POOR Px

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7
Q

Polyarteritis Nodosa

  • type of vasculitis
  • typical patient profile
  • symptoms/complications
  • important Lab Values
  • mediating Cell Type
  • Treatment
A

Medium Vessel Vasculitis

Type: Fibrosing

Patient profile: Young Adults, Strong Association with HEPATITIS B

Symptoms/complications: Episodic, Renal Fibrosis/Failure, Widespread involvement (lungs NOT affected)

Cells: neutrophils, eosinophils, and mononuclear cells

Treamtment: Fatal unless treated with Immunosuppression

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8
Q

Kawasaki Disease

  • type of vasculitis
  • typical patient profile
  • symptoms/complications
  • important Lab Values
  • mediating Cell Type
  • Treatment
A

Medium Vessel Vasculitis

Type: Fibrinoid

Patient Profile: Asian Children

Symptoms/complications: CRASH and burn

C - conjunctivitis

R - Rash

A - Adenopathy (cervicle)

S - Strawberry Tongue

H - hand or foot swelling/desquamation

burn - fever for more than 5 days

Untreated patients may experience Aneurysms and Myocardial Infarctions

Labs: Not Stated

Mediating cell type: Not Stated

Treatment: IV Ig and Asprin

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9
Q

Buerger Disease

  • type of vasculitis
  • typical patient profile
  • symptoms/complications
  • important Lab Values
  • mediating Cell Type
  • Treatment
A

Medium Vessel Vasculitis

Type: Microabscess Forming

Patient Profile: SMOKERS

Symptoms/complications: Inflammation and thrombosis in arteries of hands and feet eventually causing Cold Sensitivity, Pain, Gangrene of Digits

Labs: none stated

Treatment: QUIT SMOKING (still may not reverse symptoms)

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10
Q

Wegener Granulomatosis (aka Granulomatosis with Polyangiitis)

  • type of vasculitis
  • typical patient profile
  • symptoms/complications
  • important Lab Values
  • mediating Cell Type
  • Treatment
A

Small Vessel Vasculitis

Type: Granuloma Forming (poorly formed)

Patient Profile: Childen and Adolescents

Symptoms/complications: Chonic Sinusitis, Nasal Septal Perforation, Hemoptysis, Hematuria

Triad:

• Granulomas

• Vasculitis

• Glomerulonephritis

Labs: c-ANCA (PR3-ANCA)

Mediating Cell type: Neutrophils that have directly been activated by c-ANCA

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11
Q

Microscopic Polyangiitis

  • type of vasculitis
  • typical patient profile
  • symptoms/complications
  • important Lab Values
  • mediating Cell Type
  • Treatment
A

Small Vessel Vasculitis

Type: Leukocytoclastic Vasculitis (NO GRANULOMAS)

Patient Profile: none stated

Symptoms/complications:

• PALAPABLE PURPURA

• Lung and Kidney disease

Labs: p-ANCA present

Treatment: none stated

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12
Q

Churg-Strauss Syndrome

  • type of vasculitis
  • typical patient profile
  • symptoms/complications
  • important Lab Values
  • mediating Cell Type
  • Treatment
A

Small Vessel Vasculitis

Type: Granulomas with lots of eosinophils

Patient Profile: People with Allergies and Asthma

Symptoms/complication: mutliple organ damage but mostly asthma, allergic rhinitis, sinusitis, potential peripheral neuropathy, MYOCARDIOMYOPATHY kills 1/2 of pts.

Labs: eosinophilia and MPO-ANCA

Treatment: none stated

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13
Q

Henoch-Schonlein Purpura

  • type of vasculitis
  • typical patient profile
  • symptoms/complications
  • important Lab Values
  • mediating Cell Type
  • Treatment
A

Type: leukocytoclastic vasculitis

Patient Profile: Children with recent URI (upper respiratory tract infection)

Symptoms/complications: (tetrad)

  • Palpable Purpura - on Legs and Buttocks
  • Arthritis
  • Abdominal Pain - and possible bloody stool, and intussusception
  • Renal disease - renal vessel involvment

Labs: IgA immune complex deposition

Mediating Cell Type: plasma cells

Treatment: none stated

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14
Q

Cyroglobulinemia

  • typical patient profile
  • symptoms/complications
  • important Lab Values
A

Patient Profile: Adults with Hepatitis C

Symptoms/complications: digital ischemia leading to necrosis

Labs: proteins that precipitate from serum in the cold

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15
Q

P-ANCA

A

a

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16
Q

C-ANCA

A

a

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17
Q

What are some of the symptoms of vasculitis?

A

• Non-Specific Symptoms of Inflammation (fever, weight loss, etc.) • Palpable Purpura • Livedo Reticularis • Urticaria

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18
Q

What is polymyalgia rheumatica?

• What condition is associated with polymyalgia rheumatica?

A

Polymyalgia Rheumatica:

  • Proximal muscles aches and Stiffness
  • Elevated ESR (often >100)
  • Rapid Resolution with Steriods

Dissease Association: Termporal (giant cell) Arteritis

19
Q

****What is this? • What if you push on it and it doesn’t turn white?

A

• Palpable Purpura - NON-BLANCHING HEMORRHAGES

20
Q

****What is this? • what do you think it feels like?

A

• Urticaria - very itchy wheals

21
Q

****What is this?

A

• LIVEDO RETICULARIS - network-pattern with purplish discoloration of skin due to blood vessel dilation

22
Q

What are Palpable Purpura?

A

• Non-blanching Hemorrhages • May lead to Focal Skin necrosis and ulceration

23
Q

What is Livido Reticularis?

A

• Web-like pattern of purple colored skin due to dilation of blood vessels

24
Q

What are the two common pathologic mechanisms of vasculitis?

A
  1. IMMUNE MEDIATED inflammation and Direct invasion of vascular walls by infectious pathogens 2. Infections can also be cause immune complex formation or formation of cross-reactive antibodies
25
\*\*\*\*Differential? * 63 y/o white female experiences jaw pain when chewing. * Key Histological Features
Differential: Takayasu Arteritis and Temporal (Giant Cell) arteritis Dx: Termporal (Giant Cell) Arteritis, **granulomatous inflammation** in a **white female** over **50** Histo: **Inflammation** of Vessel Wall, Disruption of internal elastic lamina, **Intimal Fibrosis**, **GIANT CELLS, Lymphocytes in adventitia**
26
\*\*\*\*Histological Section showed a Granulomatous vasculitis. • DDx? Px?
Differential: Temporal (giant cell) Granulomatous and Takayasu Arteritis (pulseless disease) Dx: **Takayasu Arteritis** - these patient often have problems at the branchpoints of their **aortic arch** Px: **Poor Prognosis**
27
What is an important way to differential between Polyarteritis Nodosa and Wegners Granulomatosis?
Wegners Characteristically involves the lungs, however Polyarteritis Nodosa **(PAN) NEVER INVOLVES THE LUNGS**
28
\*\*\*\*Young adult presents with hepatitis B and vasculitis that comes and goes. • Dx and **KEY HISTOLOGICAL FEATURES** to look for
Dx: Polyarteritis Nodosa Histo: * **TRANSMURAL necrosis and inflammation** * **FIBRINOID NECROSIS** replaces inflammatory infiltrate caused by eos, nucs, and mononucs. \*\*\*\*\***characteristically inflammatory stages and fibrosing stages may co-exist in the same pt.\*\*\*\***
29
What two medium vessel vasculites closely resemble each other histologically? • How do you differentiate?
Polyarteritis nodosa - often in young adult with hep B Kawasaki Disease - mostly seen in young children \*\*Note: both can lead to aneurysm if untreated; Kawasaki Disease may also lead to MI.
30
\*\*\*\*Histological section taken from the finger of a smoker. • Key features, Dx?
Key Features: * **Inflammation** and Luminal Thrombosis * **Thrombosis** may contain **Microabscesses of Neutrophils** (makes sense b/c smoking raises neutrophil count) * Organization and Recanalization may occur Dx: Buerger Disease aka Thromboantiitis Obliterans
31
**Raynaud Phenomenon** * typical patient profile * symptoms/complications * Treatment
Medium Vessel Vasculitis ## Footnote Type: Young Women Symptoms/Complications: **• Pain in hands or Feet** in response to **cold or stress** **•** May cause **cyanosis or erythema** Treatment: **calcium channel blockers and avoid the cold**
32
\*\*\*What shown here?
• Raynaud Phenomenon
33
\*\*\*\*What type of vasculitis does this girl probably have?
Wegner's Granulomatosis - can cause nasal perforation and is typically found in children and adolescents
34
\*\*\*\*\*Shown here is a lung? • Key Histological Features, Dx?
Dx: Wegner's Granulomatosis Key Features: * Necrosis of Lung with Eosinophils, Multinucleated **Giant cells** (seen at top right) * **Granulomas** surrounded by **Pallisading Histiocytes** (star marks this) with central necrosis
35
What two diseases may closely resemble Microscopic Polyangiitis and how do you differentiate?
Wegener's Granulomatosis and PAN ## Footnote Wegener's - has granuloma formation, **Microscopic Polyangiitis has NO GRANULOMAS** PAN - has lesions of all different ages, **Microscopic Polangiitis has lesions that are ALL THE SAME AGE**
36
What are the P's of Microscopic Polyangiitis?
**• Polyangiitis, mPo-ANCA, Palpable Purpura**
37
\*\*\*\*\*What are some key histological features in this person who expresses p-ANCA?
Dx: Microscopic Key Histological Features: Leukocytoclastic Vasculitis with **Fragmention of Neutrophils in and around Vesssel Walls.**
38
\*\*\*\*\*This is a vasculitis, what type?
Churg-Strauss Syndrome • Resembles PAN and Microscopic Polyangiitis BUT includes **GRANULOMAS** with **EOSINOPHILS**
39
What clinical triad is can help you to diagnose Behcet Disease?
* Recurrent Oral Aphthous Ulcers * Genital Ulcers * Uveitis
40
**Behcet Disease** * Type * Epidemiology * Treatment
Type: Leukocytoclastic Vasculitis and Pnniculitis Epidemiology: Most common in Japan, China, and Mediterranean Treatment: Steroids or TNF agonists for immunosuppression \*\*\*Ulcers may affect eyes, feet, and genitals (can't see, can't pee, can't climb a tree)
41
\*\*\*\*\*What disease is this? • Key histological Features
Behcet Disease Key Features: Endothelial Swelling (black arrow) and Perivascular inflammatory cell infiltrate
42
What are some of the acute and chronic effects of Henoch-Schonlein Purpura?
Acute: • **IgA immune complexes** deposit into the vessels causing **glomerulonephritis** Chronic: • **Glomerular Necrosis** and **Hyaline Ateriolosclerosis**
43
**Infectious Vasculitis** * Bugs responsible * complications
Bugs: Pseudomonas, Aspergillis, and Mucor species Complications: Mycotic aneurysms, Thrombosis, and infarction
44
\*\*\*\*What is wrong with this aorta?
* Wall is fibrotic * Mural Absess with lots of neutrophil infiltration can be seen