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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

A
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
Q

IgA vasculitis

A
  • 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
Q

Diagnosing IgA vasculitis

A

Biopsy of area and direct immunofluorescence against IgA

27
Q

Cryoglobulinemic vasculitis

A
  • 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
Q

Cryoglobulin

A

Igs characterized by their ability to precipitate at low temperatures

29
Q

Acryocyanosis

A

Bluish or purple-coloring of the hands or feet caused by low circulation

30
Q

Diagnosis of cryoglobulinemic vasculitis

A
  • 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
Q

Giant cell arteritis

A
  • 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
Q

Polymyalgia rheumatica

A
  • 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
Q
A
  • 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
Q

Immune cell infiltrate characteristics in GCA

A
  • 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
Q

Giant cell arteritis diagram

A
36
Q

CD4 T cell polarity

A
37
Q

Th17 polarization diagram

A
38
Q

Digital ischemia may be a presenting symptom of ___.

A

Digital ischemia may be a presenting symptom of medium vessel arteritis.

39
Q

___ is a form of vasculitis associated with high titer rheumatoid factor.

A

Cryoglobulinemia is a form of vasculitis associated with high titer rheumatoid factor.

HMS IDD (28 decks)

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