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Senior - Rhuem Chart > 9 - Vasculitis > Flashcards

9 - Vasculitis Flashcards

1
Q

Vasculitis breaks down into?

A

Large vessel
Medium vessel
Small vessel - immune-complex mediated
Small vessel - ANCA associated vasculitis

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2
Q

Large vessel includes

A

Large vessel

  • giant cell arteritis
  • Polymyalgia rheumatica
  • takaysu’s arteritis
  • behcet syndrome
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3
Q

Medium vessel includes

A

Medium vessel

  • polyarteritis nodosa
  • buerger’s disease
  • Kawaski s disease
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4
Q

Small vessel immune complex mediated vasculitis includes

A

Small vessel - immune-complex mediated

  • cutaneous (leukocytoclastic) vasculitis
  • henoch-schonlein purpura
  • cryglobulinemia
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5
Q

Small vessel ANCA-associated vasculitis includes

A

Small vessel - ANCA associated vasculitis

  • granulomatosis w polyangitis (wegners)
  • churg-strauss syndrome
  • microscopic polyangiitis
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6
Q

MC type of vasculitis in adults?

A

Giant cell arteritis (GCA)

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7
Q

Who gets GCA?

A

Female >50 yo

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8
Q

S/S of GCA?

A 
HA
Scalp tenderness
Fever
Visual symptoms 
Jaw claudication
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9
Q

GCA has a risk of?

A

Sudden blindness due to ischemic optic neuritis

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10
Q

Diagnostics for GCA?

A

ESR - very high (80-100)

Temporal artery biopsy (w/in 2 wks of steroids)

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11
Q

Tx for GCA?

A

PROMPT initiation of HIGH DOSE corticosteoids
- Prednisone until better (3-4 wks)

Low dose aspirin
- vision loss or stroke prevention

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12
Q

Dont delay steroids for?

A

The temporal artery biopsy. Start that shit

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13
Q

GCA also needs to worry about?

A

Osteoporosos

Focus on prevention

  • smoking
  • exercise
  • calcium
  • D
  • biphosphonate (BMD)
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14
Q

Age for polymyalgia rheumatica (PMR)

A

> 50

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15
Q

PMR is like ___ but ___

A

Like GCA but 2-3x more common

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16
Q

Unlike GCA PMR…

A

Has no risk for blindness with “pure PMR

But it may progress to GCR

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17
Q

S/S of PMR?

A

Stiffness + pain in shoulders and hips
- esp mornings (>30min)

“I feel old”
Muscle pain (not in joint) >1mo
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18
Q

PMR has stiffness in 2 of 3 ares:

A

Neck/torso
Shoulders
Hips

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19
Q

Diagnostics for PMR?

A

ESR > 40

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20
Q

Tx for PMR?

A

Low dose prednisone
- x 1-2 yrs

Weekly methotrexate increases success

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21
Q

Prognosis for GCA?

A

Thoracic aortic aneurysms
aortic dissection

are late complications

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22
Q

Takayasu’s arteritis is aka?

A

Pulseless disease

Occlusive thromboaortopathy

Granulomatous vasculitis of the aorta and major branches

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23
Q

Who get takayasu’s arteritis?

A

MC = asian females age 10-30

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24
Q

What distinguishes takaysu’s arteritis?

A

Aorta/branch involvement

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25
Q

Pt presentation for takayasu’s arteritis?

A

Appear chronically ill

  • fatigue
  • wt loss
  • low grade fever
  • upper extremity claudication
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26
Q

PE for takayasu’s arteritis?

A

Abnormal pulse exam

  • Unequal blood pressures
  • Carotid/subclavian bruits
  • Hypertension

Cardiac:

  • aortic regurg
  • angina
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27
Q

Labs for takayasu’s arteritis?

A

high ESR

high CRP

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28
Q

Tx for takayasu’s arteritis?

A

Prolonged corticosteroid treatment
- HIGH DOSE prednisone x 1 mo then taper 4-6 mo

+ methotrexate/mycophenolate mofetil

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29
Q

Surgery for takayasu’s arteritis?

A

Surgery/revascularization therapy

  • PCA (renal arteries)
  • surgical revascularization
  • aortic valve replacement
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30
Q

Prognosis for takayasu’s arteritis?

A

10 yr survival&raquo_space; 80-90%

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31
Q

Who get behcet syndrome?

A

Young adults 25-35
Asian
Turkish
Mid eastern

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32
Q

Genetic risk factor for behcet syndrome?

A

HLA B51

33
Q

What is unique about behcet syndrome?

A

It is an inflammatory vaasculitis that affects large, medium and small blood vessels

All 3 sizes, both arteries and veins

It gets around it you know what i mean

34
Q

If large vessels are involved (behcet syndrome)

A

Pulmonary artery aneurysm

Pulmonary hemorrhage

35
Q

Hallmark of behcet syndrome?

A

Painful aphthous ulcers in mouth

  • erythema nodosum-like lesions that ulcerate
36
Q

behcet syndrome also has? (Eyes)

A

Anterior or posterior uveitis

37
Q

Labs for behcet syndrome?

A

No specific confirmation labs

ESR/CRP often elevated

38
Q

Tx for behcet syndrome?

A

Multiple:

  • corticosteroids
  • dapsone
  • colchicine
  • azathioprine
39
Q

behcet syndrome sever ocular and CNS complication tx?

A

Pick one:

  • infliximab
  • cyclosporine
  • cyclophosphamide
40
Q

What is polyarteritis nodosa (PAN)?

A

Necrotizing inflammatory vasculitis of med arteries and muscular arterioles

41
Q

PAN spares?

A

Venous circulation and capillaries

42
Q

PAN presentation?

A

Insidious onset over weeks to months

- w/in 6 months of Hep B infection

43
Q

Presentation of PAN? (S/S)

A 
Mononeuritis multiplex (foot drop)
Freq abdominal pain (post eating)
- if mesenteric vessel involved
HTN (2/2 renal artery involv)
Derm presentatinos
44
Q

Derm presentation of PAN?

A

Palpable purpura
Livedo reticularis
Skin ulcers
Digital gangrene

45
Q

Diagnostic for PAN?

A
  • HBV testing (at diagnosis)
  • ANCA-neg(true PAN)
  • Biopsy (skin, nerve, muscle)
  • Angiography (mesenteric)
46
Q

Tx for PAN?

A

Prolonged corticosteoids
Cyclophosphamide (cytoxan)

Most achieve remission w this

47
Q

Prognosis of PAN?

A

W/o tx - 5 yr = 10%

W tx 5 yr = 60-90%

48
Q

Classic clinical finding of small vessel vasculitis?

A

Palpable purpura

49
Q

Small vessel vasculitis includes?

A

Arterioles
Venules
Capillaries

50
Q

MC vasculitis in kids <6yo?

A

Henoch-schonlein purpura (HSP)

51
Q

HSP is strongly associated with?

A

IgA deposition

52
Q

HSP usually follows?

A 
Viral URI
But also:
- EBV
- Varicella
- Parvovirus
- Strep
- Staph
- Mycoplasm
53
Q

S/s of HSP?

A

Palpable purpura (lower extremeties)
Arthralgias/arthritis
GI - colocky abd pain
Renal - IgA immune complex deposition -> glomerulonephritis

54
Q

Diagnostic for HSP?

A

Clinical symptoms

Biopsy w predominant IgA deposition

55
Q

Mandatory finding for HSP?

A

Palpable purpuric rash

56
Q

Tx for HSP?

A

Supportive care

Tylenol/NSAIDs

57
Q

Cryoglobulinemia caused by?

A

Immunoglobulins (antibodies IgG, IgM) that precipate out of blood at low temp (<37*C)

58
Q

Cryoglobulinemia is associated with?

A

Hep C infection

59
Q

Types of Cryoglobulinemia?

A

Type I - hematologic syndrome
- hyperviscosity
Type II and III - can cause small vessel vasculitis

60
Q

Clinical triad of Cryoglobulinemia?

A

Purpura
Arthralgias
Myalgias

Also includes peripherial neuropathy
And renal disease

61
Q

Cryoglobulinemia lab work?

A

RF POS

Cryoglobulins in conjunction

62
Q

Tx of Cryoglobulinemia?

A

Treat the Hep C

  • sofosburiv
  • simeprevir
  • harvoni

Severe cases get steroids or immunosuppressive agents (ESRD)

63
Q

Granulomatosis with polyangitis (GPA) is a?

A

Small vessel ANCA associated vasculitis

A necrotizing vasculitis

64
Q

GPA is aka?

A

Wegener’s

65
Q

Who gets wegeners?

A

White N. Europeans in 40-50’s

66
Q

GPA (wegner’s) triad?

A

Manifestations of:

  • Upper respiratory
  • Lower respiratory
  • Renal
67
Q

Hallmark of GPA (wegner’s)?

A
  • Granulomatous inflammation
  • Vasculitis
  • Necrosis
68
Q

Upper respiratory disease with GPA (wegner’s)?

A

Nasal manifestations

Sinus pain
Otitis media
Subglottic stenosis
Epistaxis
Persistent rhinorrhea
Septal perforation
Saddle nose
69
Q

Lower respiratory disease GPA (wegner’s)?

A
  • Alveolar capillaritis resulting in hemoptysis
  • Granulomatous nodules and caveties
  • Glomerulonephritis&raquo_space; renal involvement
70
Q

Diagnostics for GPA (wegner’s)?

A

C-ANCA positive (unlike CSS)

Absent pANCA (less useful)

Biopsy showing granulomatous inflammation
UA - RBC casts and hematuria

CT>CXR

71
Q

Tx for GPA (wegner’s)?

A

1st line:
- corticosteroids/cyclophosphamide or ruximab

Post remission:
- azathioprine/methotrexate

72
Q

Churg strauss syndrome is aka?

A

Eosinophilic granulomatosis with polyangiitis

73
Q

What is churg-strauss syndrome?

A

Necrotizing vasculitis associated with

  • allergic rhinitis
  • reactive airway disease
  • peripheral eosinophilia
74
Q

Clinical findings for churg-strauss syndrome?

A

Allergic rhinitis + nasal polyps

Adult onset asthma

75
Q

Clinical phases of churg-strauss syndrome?

A

Prodromal phase: allergic rhinitis or asthma

Eosinophilic phase
- peripheral blood eosinophilia and eisinophilic infiltration of multiple organs

Vasculitic phase:

  • life threatening systemic necrotizing vasculitis of small and med vessels
  • multi organs
76
Q

churg-strauss syndrome does not have ?

A

Renal involvment (vs GPA)

77
Q

labs for churg-strauss syndrrome?

A

P-ANCA pos (vs GPA)

Biopsy
Vascular
Extravascular (nerve, muscle, heart)
Typical clinical features:
- asthma
- allergic rhinitis
- eosinophilia
78
Q

Tx for churg-strauss syndrome?

A

Corticosteroids
Cyclophosphamatide for neuropathy/glomerulonephritis

Azathioprine/Methotrexate can be added to corticosteroids

Senior - Rhuem Chart (10 decks)

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