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Cardio - Gupta > Vasculitis > Flashcards

Vasculitis Flashcards

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
Q

****Differential?

  • 63 y/o white female experiences jaw pain when chewing.
  • Key Histological Features
A

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
Q

****Histological Section showed a Granulomatous vasculitis.

• DDx? Px?

A

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
Q

What is an important way to differential between Polyarteritis Nodosa and Wegners Granulomatosis?

A

Wegners Characteristically involves the lungs, however Polyarteritis Nodosa (PAN) NEVER INVOLVES THE LUNGS

28
Q

****Young adult presents with hepatitis B and vasculitis that comes and goes.

• Dx and KEY HISTOLOGICAL FEATURES to look for

A

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
Q

What two medium vessel vasculites closely resemble each other histologically?

• How do you differentiate?

A

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
Q

****Histological section taken from the finger of a smoker.

• Key features, Dx?

A

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
Q

Raynaud Phenomenon

  • typical patient profile
  • symptoms/complications
  • Treatment
A

Medium Vessel Vasculitis

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
Q

***What shown here?

A

• Raynaud Phenomenon

33
Q

****What type of vasculitis does this girl probably have?

A

Wegner’s Granulomatosis - can cause nasal perforation and is typically found in children and adolescents

34
Q

*****Shown here is a lung?

• Key Histological Features, Dx?

A

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
Q

What two diseases may closely resemble Microscopic Polyangiitis and how do you differentiate?

A

Wegener’s Granulomatosis and PAN

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
Q

What are the P’s of Microscopic Polyangiitis?

A

• Polyangiitis, mPo-ANCA, Palpable Purpura

37
Q

*****What are some key histological features in this person who expresses p-ANCA?

A

Dx: Microscopic

Key Histological Features: Leukocytoclastic Vasculitis with Fragmention of Neutrophils in and around Vesssel Walls.

38
Q

*****This is a vasculitis, what type?

A

Churg-Strauss Syndrome

• Resembles PAN and Microscopic Polyangiitis BUT includes GRANULOMAS with EOSINOPHILS

39
Q

What clinical triad is can help you to diagnose Behcet Disease?

A
  • Recurrent Oral Aphthous Ulcers
  • Genital Ulcers
  • Uveitis
40
Q

Behcet Disease

  • Type
  • Epidemiology
  • Treatment
A

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
Q

*****What disease is this?

• Key histological Features

A

Behcet Disease

Key Features: Endothelial Swelling (black arrow) and Perivascular inflammatory cell infiltrate

42
Q

What are some of the acute and chronic effects of Henoch-Schonlein Purpura?

A

Acute:

• IgA immune complexes deposit into the vessels causing glomerulonephritis

Chronic:

• Glomerular Necrosis and Hyaline Ateriolosclerosis

43
Q

Infectious Vasculitis

  • Bugs responsible
  • complications
A

Bugs: Pseudomonas, Aspergillis, and Mucor species

Complications: Mycotic aneurysms, Thrombosis, and infarction

44
Q

****What is wrong with this aorta?

A
  • Wall is fibrotic
  • Mural Absess with lots of neutrophil infiltration can be seen

Cardio - Gupta (12 decks)

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