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Vasculitis Flashcards

1
Q

vasculitis - defined

A

*inflammation of the blood vessels
*causes destruction of the vessel integrity:
-narrowed artery, organ ischemia, aneurysm / hemorrhage, thrombus
*multi-system
*also associated with inflammation outside vessel

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2
Q

general symptoms to suggest vasculitis

A

*fever
*weight loss
*anorexia
*malaise
*failure to thrive
*evidence of end-organ damage

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3
Q

more specific symptoms that may suggest vasculitis

A

*hemoptysis
*regional ischemia
*glomerulonephritis
*rash (palpable purpura, ulceration)
*mononeuritis multiplex
*bruits
*arthritis/arthralgia

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4
Q

examples of large-vessel vasculitis

A

*giant cell arteritis
*polymyalgia rheumatica
*Takayasu arteritis

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5
Q

examples of medium-vessel vasculitis

A

*polyarteritis nodosa
*Kawasaki disease

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6
Q

examples of small-vessel vasculitis

A

*granulomatosis with polyangiitis (GPA)
*microscopic polyangiitis (MPA)
*eosinophilic granulomatosis with polyangiitis (EGPA)
*IgA vasculitis
*cryoglobulinemia

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7
Q

giant cell arteritis - epidemiology

A

*age: > 50 yo
*prevalence increases with age
*females > males (2:1)

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8
Q

giant cell arteritis - symptoms

A

*systemic symptoms: fever, weight loss, fatigue
*temporal headaches
*scalp tenderness
*jaw claudication
*changes in vision (amaurosis fugax, diplopia, visual loss; if missed, can lead to blindness)
*can be associated with PMR (40-50%)

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9
Q

giant cell arteritis - diagnosis

A

*biopsy of the temporal artery:
-granulomatous inflammation
-multinucleated giant cells
-predominantly involves media and elastic lamina fragmentation
*if one side negative and suspicion high, biopsy the contralateral side
*often have an elevated ESR

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10
Q

giant cell arteritis - treatment

A

*high dose steroids (prednisone) before anything else (including before biopsy)
*steroids do not effect the biopsy results for a few weeks (try to biopsy within 7 days of starting steroids)

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11
Q

giant cell arteritis - new advances

A
  1. imaging:
    -temporal artery ultrasound
    -PET imaging
  2. medications:
    -methotrexate has been used historically
    -toclizumab (IL-6 inhibitor)
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12
Q

giant cell arteritis - associated with what other condition?

A

*polymyalgia rheumatica

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13
Q

giant cell arteritis - important complication

A

*can develop permanent vision loss (blindness) if left untreated

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14
Q

polymyalgia rheumatica (PMR) - epidemiology

A

*age > 50 yo
*prevalence increases with age

recall: associated with giant cell arteritis

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15
Q

polymyalgia rheumatica (PMR) - symptoms

A

1. PAIN & stiffness in symmetric proximal muscles (shoulder and hip girdle muscles)
2. ABSENCE OF TRUE WEAKNESS (stop because it hurts)
*elevated ESR or CRP
*diagnosis = clinical
*associated with giant cell arteritis

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16
Q

polymyalgia rheumatica (PMR) - treatment

A

*low dose prednisone
*improves almost overnight

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17
Q

overlap between giant cell arteritis (GCA) and polymyalgia rheumatica (PMR)

A

*40% of pts with GCA will have PMR
*15% of pts with PMR will have GCA
*ALWAYS screen for the other during each visit

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18
Q

Takayasu’s arteritis - epidemiology

A

*vasculitis of aorta and major branches
*fairly rare
*YOUNG ( < 40 yo)
*female > male (8:1)
*Asian descent (highest prevalence)

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19
Q

Takayasu’s arteritis - symptoms

A

*vasculitis of aorta and major branches
*fever, fatigue, weight loss
* upper extremity claudication (classically, young female swimmer having trouble finishing laps b/c arms hurt)
*“pulseless disease” = absent or weak upper extremity pulses
*elevated ESP/CRP
*HTN (renal artery stenosis)

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20
Q

Takayasu’s arteritis - physical exam

A

*bruits over carotid, subclavian, or renal
*may become pulseless (late)
*discordant blood pressure from one arm to the other

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21
Q

Takayasu’s arteritis - diagnosis

A

*angiography (conventional, CTA, or MRA)
*biopsy is not generally feasible but would show granulomatous inflammation

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22
Q

Takayasu’s arteritis - treatment

A

*high dose steroids + steroid sparing agent

23
Q

polyarteritis nodosa (PAN) - epidemiology

A

*age: 40-60 yo
*seen in children as well
*slight male predominance
*often associated with Hepatitis B

24
Q

polyarteritis nodosa (PAN) - pathogenesis

A

*immune complex mediated (type III hypersensitivity reaction)

25
polyarteritis nodosa (PAN) - symptoms
***fever, arthralgia, weight loss *peripheral nerve involvement** (mononeuritis multiplex) *renal involvement (hypertension) *testicular pain *palpable purpura ***micro-aneurismal formation: -abdominal pain** / intestinal angina **overall: affects the skin, kidneys, intestines, and nerves; SPARES the LUNGS**
26
polyarteritis nodosa (PAN) - diagnosis
*biopsy of skin, muscle or sural nerve: -shows **transmural inflammation and fibrinoid necrosis** *abdominal angiogram showing vasculitis
27
polyarteritis nodosa (PAN) - treatment
*if Hep B positive: high dose steroids and antivirals *if Hep B negative: high dose steroids and immunosuppression
28
Kawasaki disease - epidemiology
*pediatric disease typically (**children < 5 years old**) *rare in adults (consider HIV if present in adults) *aka mucocutaneous lymph node syndrome
29
Kawasaki disease - symptoms
***FEVER > 5 days** (required for dx) *conjunctivitis *diffuse erythematous rash *oral mucositis, **Strawberry tongue** ***rash on palms & soles**, desquamating *complications: -acute coronary syndrome -coronary artery aneurysms
30
Kawasaki disease - important complication
*can develop **coronary artery aneurysms**
31
Kawasaki disease - treatment
***high dose salicylates (aspirin) *IVIG** *echo to evaluate for aneurysms (initially + 2 weeks + 6-8 weeks)
32
ANCA vasculitis
*small vessel vasculitis *p-ANCA = MPO *c-ANCA = PR3
33
granulomatosis with polyangiitis (GPA) - symptoms
*upper & lower respiratory tract + kidneys *sinusitis, nasal, or ear involvement; **saddle nose deformity** *cough, hemoptysis, pleurisy; cavitary lesions, often diagnosed with "multifocal pneumonia" *pauci-immune glomerulonephritis (RBCs, casts, protein) *eye involvement = uveitis *palpable purpura
34
granulomatosis with polyangiitis (GPA) - diagnosis
*clinical history *lung or kidney biopsy: -granulomas in tissue -pauci-immune glomerulonephritis ***c-ANCA, anti-PR3+**
35
granulomatosis with polyangiitis (GPA) - treatment
*high dose steroids *cyclophosphamide or rituximab
36
microscopic polyangiitis (MPA) - symptoms
***lower respiratory tract + kidneys (no upper airway involvement)** *fever, arthralgia, palpable purpura *rapidly progressive glomerulonephritis (pauci-immune) *pulmonary hemorrhage
37
microscopic polyangiitis (MPA) - diagnosis
*biopsy skin, lung, or kidney **(necrotizing but not granulomas) *p-ANCA, anti-MPO+**
38
microscopic polyangiitis (MPA) - treatment
*high dose steroids, cyclophosphamide or rituximab *relapse rate higher than GPA
39
eosinophilic granulomatosis with polyangiitis (EGPA) - symptoms
***history of asthma or allergic rhinitis** (may develop as adult) *multi-organ involvement possible *lung, skin, heart, and nerves: -pulmonary infiltrates -palpable purpura -mononeuritis multiplex
40
eosinophilic granulomatosis with polyangiitis (EGPA) - diagnosis
*biopsy: granulomatous, necrotizing vasculitis with **eosinophilic tissue involvement** ***p-ANCA + (anti-MPO)** (only in 40%) *peripheral eosinophilia (>10%)
41
eosinophilic granulomatosis with polyangiitis (EGPA) - treatment
*high dose steroids *maybe cyclophosphamide or rituximab if severe
42
IgA vasculitis - epidemiology
*previously Henoch-Schonlein Purpura *most common **CHILDHOOD vasculitis**: -ages 3 to 15 years; **peak = 4-6 years old** -males > females (2:1) -30 to 50% have a **preceding URI** *rare in adults (associated with malignancy; more renal involvement)
43
IgA vasculitis - clinical presentation
TETRAD: 1. **purpura or petechiae** with lower limb predominance 2. **arthritis or arthralgias** (large joints, lower extremity, oligoarticular) 3. **abdominal pain** (periumbilical pain, N/V, intussusception) 4. **renal involvement** (hematuria or proteinuria)
44
IgA vasculitis - treatment
*supportive *NSAIDs *prednisone if severe renal involvement
45
Behcet's syndrome
*a small vessel vasculitis *common in **Mediterranean descent *oral and genital ulcerations** *other manifestations: uveitis, rash, pulmonary hemorrhage
46
types of cryoglobulins
*type 1: monoclonal IgM or IgG; associated with lympho-, myeloproliferative disease *type 2: monoclonal IgM RF plus polyclonal IgG *type 3: polyclonal IgM RF plus polyclonal IgG ***"mixed" cryoglobulins = types 2 & 3:** -type 2 = associated with Hep C -type 3 = associated with autoimmune disease
47
type 2 cryoglobulinemic vasculitis - associated with...
**hepatitis C**
48
type 3 cryoglobulinemic vasculitis - associated with...
autoimmune disease
49
cryoglobulinemic vasculitis - symptoms
*** purpura** - lower extremity/dependent areas ***mononeuritis multiplex** *immune complex **glomerulonephritis**
50
cryoglobulinemic vasculitis - labs
*low complements (C4) *Hep B, Hep C, HIV *RF + but negative CCP
51
treatment of cryoglobulinemic vasculitis
*Hep C associated (type 2): treat hep C, short course steroids if severe manifestations *non Hep C associated: rituximab or cyclophosphamide
52
cocaine-induced vasculitis
*** levamisole** is causative agent (70% of US cocaine contaminated) ***skin necrosis** is predominate feature: **ear lobe or nose necrosis** *clinically looks a lot like GPA, but worse cutaneous destruction, decreased systemic features
53
cocaine-induced vasculitis - labs
*p-ANCA positive *MPO negative *can be PR3 positive *anti-human neutrophil elastase positive *agranulocytosis
54
cocaine-induced vasculitis - treatment
*supportive *stop cocaine

Rheumatology Exam (20 decks)

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