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HMS IDD > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Types of vasculitis are organized by. . .

A

. . . the size of the blood vessels affected by the pathology

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2
Q

Vasculitis diagram: Where is the inflammation?

A
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3
Q

“Large” blood vessels

A

The aorta and its major branches

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4
Q

Medium blood vessels

A

Visible to the naked eye or by angiography, often named arteries that branch off of the “major” central arteries.

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5
Q

Small blood vessels

A

Arterioles, capillaries, and venules

Microscopic

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6
Q

Etiologies of large vessel vasculitis

A
  • Giant cell arteritis
  • Takayasu arteritis
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7
Q

Etiologies of medium vessel vasculitis

A
  • Polyarteritis Nodosa
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8
Q

Etiologies of small vessel vasculitis

A
  • ANCA-associated vasculitis
  • Cryoglobulinemic vasculitis
  • IgA vasculitis
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9
Q

Organs selectively affected by small vessel vasculitis and corresponding symptoms

A
  • skin - purpura
  • nerves - neuropathy
  • lungs - coughing, nodules, hemoptysis
  • kidneys - hematuria
  • joints - synovitis, arthritis, arthralgia
  • eyes - inflammatory symptoms
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10
Q

Organs affected by medium vessel vasculitis and associated symptoms

A
  • Mesenteric - abdominal pain
  • Renal - kidney infarct, kidney pain
  • Nerves - neuropathy
  • Skin - ulcers, livedo, gangrene
  • associated w/ insidious presentation
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11
Q
A

Livedo

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12
Q

Organs affected by large vessel vasculitis and associated symptoms

A
  • Brain - headache
  • Eyes - blindness
  • Muscles of face - Jaw claudication
  • Fever
  • Extremity claudication
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13
Q

Specific lab tests are available to test for ___.

A

Specific lab tests are available to test for small vessel vasculitis, but not medium or large vessel vasculitis.

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14
Q

Small vessel vasculitis may be due to an underlying ___.

A

Small vessel vasculitis may be due to an underlying connective tissue disease.

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15
Q

Tests for small vessel vasculitis

A
  • Usually involve testing for glomerulonephritis
  • Test for proteinuria
  • Stain H and E for RBC casts
  • Kidney biopsy for “crescent” of immune cells surrounding a glomerulus
  • Full-body observation for purpura
  • CT scan for diffuse alveolar hemorrhage
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16
Q

Leukocytoclastic vasculitis

A
  • When white blood cells attack blood vessels
  • General term for any small-vessel vasculitis
17
Q
A

Small vesse vasculitis on H and E. Note the asterix in the vessel wall and extravasated RBCs (the histological equivalent to the finding of non-blanching erythema)

18
Q

Asterix

A

Firboid necrosis in a vessel wall

19
Q

Anti-neutrophil cytoplasmic antibodies (ANCA)

A
  • Autoreactive antibodies against myeloperoxidase and proteinase-3
  • Known to cause glomerulonephritis
20
Q

Indirect immunofluorescence test for ANCA

A
  • Anti-MPO stains perinuclearly (pANCA)
  • Anti-PR3 stains cytoplasmically (cANCA)
  • Minor ANCAs such as elastase, Lamp-2, or lactoferrin may confound results (usually pANCA)
  • ELISA used to confirm following indirect immunofluorescence screening test
21
Q

Three phenotypes of ANCA

A
  • Microscopy polyangitis (MPA)
    • MPO > PR3, necrotizing vasculitis
  • Granulomatosis with polyangitis (GPA)
    • PR3 > MPO, necrotizing vasculitis, granulomas
  • Eosinophilic granulomatosis with polyangitis (EGPA)
    • MPO only, necrotizing vasculitis, granulomas, eosinophilia
22
Q

____ is central to ANCA

A

NETosis is central to ANCA

23
Q

NETosis model for ANCA

24
Q

Role of DNAse I in ANCA

A

Impaired DNAse I activity leads to NET persistence following physiological NETosis. This leads to some damage which is then associated with anti-PR3 and anti-MPO antibody development. These ANCAs then activate further NETosis via FcγRI binding.

The cycle goes on.

25
IgA vasculitis
* Non-ANCA-mediated small cell vasculitis, rather IgA immune complex mediated * Generally preceded by the initiaton of a new medication (often antibiotics) * General presentation of **purpura, arthralgia/peri-arthralgia, abdominal pain (from interstitial vasculitis), glomerulonephritis**
26
Diagnosing IgA vasculitis
Biopsy of area and direct immunofluorescence against IgA
27
Cryoglobulinemic vasculitis
* Characterized by overabundance of cryoglobulins * May be primary, or may be secondary to: malignancy, connective tissue disease (steven-johnson syndrome), or Hep C infection * **Meltzer's triad: purpura, arthralgias, myalgias** * Membranoproliferative glomerulonephritis presenting as hematuria * Peripheral symmetric polyneuropathy (often painful) * Reynaud's phenomenon and acryocyanosis
28
Cryoglobulin
Igs characterized by their ability to precipitate at low temperatures
29
Acryocyanosis
Bluish or purple-coloring of the hands or feet caused by low circulation
30
Diagnosis of cryoglobulinemic vasculitis
* Serum cryoglobulin measurement (must be collected in warm serum tube) * Hypocomplementemia (C4 depressed out of proportion to C3, due to C4 depletion) * High-titer Rheumatoid factor * Serum protein electrophoresis * Serum free light chain measurement
31
Giant cell arteritis
* Form of large vessel vasculitis * Most common vasculitis in adults * Occurs more frequently w/ older age, almost all cases \>50 yr old * Majority of patients female Caucasian (due to HLA clustering) * Distinct clinical phenotypes * Headache, scalp tenderness, visual loss, jaw claudication * Visible superficial arteries with pulsation (often temporal artery) * Fever of unknown origin (likelihood increases w/ age) * Polymyalgia rheumatica (widespread myalgia, aches, and flu-like symptoms)
32
Polymyalgia rheumatica
* Associated w/ giant cell arteritis (obsv. in 50% of GCA patients, but only 15-20% of PMR patients have GCA) * 2nd most common rheumatic disease in elderly * Symmetric \>30 min morning stiffness and aching pain in neck, shoulders, hips * Mostly due to bursitis, synovitis may be associated
33
* H and E of giant cell arteritis * Characteristics: * Trans-mural mononuclear inflammation (mostly MΦ and CD4) * Occasional multinucleated giant cells * Predilection for internal elastic lamina * Intimal hyperplasia
34
Immune cell infiltrate characteristics in GCA
* Dominated by oligoclonal CD4 T cells * Th1 and Th17 polarization * Assocaition w/ HLA-DRB1\*04 (note that this is the same family of HLA genes that predisposes to rheumatoid arthritis)
35
Giant cell arteritis diagram
36
CD4 T cell polarity
37
Th17 polarization diagram
38
Digital ischemia may be a presenting symptom of \_\_\_.
Digital ischemia may be a presenting symptom of **medium vessel arteritis**.
39
\_\_\_ is a form of vasculitis associated with high titer rheumatoid factor.
**Cryoglobulinemia** is a form of vasculitis associated with high titer rheumatoid factor.

HMS IDD (28 decks)

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