Vasculitis Flashcards
vasculitis - defined
*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
general symptoms to suggest vasculitis
*fever
*weight loss
*anorexia
*malaise
*failure to thrive
*evidence of end-organ damage
more specific symptoms that may suggest vasculitis
*hemoptysis
*regional ischemia
*glomerulonephritis
*rash (palpable purpura, ulceration)
*mononeuritis multiplex
*bruits
*arthritis/arthralgia
examples of large-vessel vasculitis
*giant cell arteritis
*polymyalgia rheumatica
*Takayasu arteritis
examples of medium-vessel vasculitis
*polyarteritis nodosa
*Kawasaki disease
examples of small-vessel vasculitis
*granulomatosis with polyangiitis (GPA)
*microscopic polyangiitis (MPA)
*eosinophilic granulomatosis with polyangiitis (EGPA)
*IgA vasculitis
*cryoglobulinemia
giant cell arteritis - epidemiology
*age: > 50 yo
*prevalence increases with age
*females > males (2:1)
giant cell arteritis - symptoms
*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%)
giant cell arteritis - diagnosis
*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
giant cell arteritis - treatment
*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)
giant cell arteritis - new advances
- imaging:
-temporal artery ultrasound
-PET imaging - medications:
-methotrexate has been used historically
-toclizumab (IL-6 inhibitor)
giant cell arteritis - associated with what other condition?
*polymyalgia rheumatica
giant cell arteritis - important complication
*can develop permanent vision loss (blindness) if left untreated
polymyalgia rheumatica (PMR) - epidemiology
*age > 50 yo
*prevalence increases with age
recall: associated with giant cell arteritis
polymyalgia rheumatica (PMR) - symptoms
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
polymyalgia rheumatica (PMR) - treatment
*low dose prednisone
*improves almost overnight
overlap between giant cell arteritis (GCA) and polymyalgia rheumatica (PMR)
*40% of pts with GCA will have PMR
*15% of pts with PMR will have GCA
*ALWAYS screen for the other during each visit
Takayasu’s arteritis - epidemiology
*vasculitis of aorta and major branches
*fairly rare
*YOUNG ( < 40 yo)
*female > male (8:1)
*Asian descent (highest prevalence)
Takayasu’s arteritis - symptoms
*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)
Takayasu’s arteritis - physical exam
*bruits over carotid, subclavian, or renal
*may become pulseless (late)
*discordant blood pressure from one arm to the other
Takayasu’s arteritis - diagnosis
*angiography (conventional, CTA, or MRA)
*biopsy is not generally feasible but would show granulomatous inflammation
Takayasu’s arteritis - treatment
*high dose steroids + steroid sparing agent
polyarteritis nodosa (PAN) - epidemiology
*age: 40-60 yo
*seen in children as well
*slight male predominance
*often associated with Hepatitis B
polyarteritis nodosa (PAN) - pathogenesis
*immune complex mediated (type III hypersensitivity reaction)
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
polyarteritis nodosa (PAN) - diagnosis
*biopsy of skin, muscle or sural nerve:
-shows transmural inflammation and fibrinoid necrosis
*abdominal angiogram showing vasculitis
polyarteritis nodosa (PAN) - treatment
*if Hep B positive: high dose steroids and antivirals
*if Hep B negative: high dose steroids and immunosuppression
Kawasaki disease - epidemiology
*pediatric disease typically (children < 5 years old)
*rare in adults (consider HIV if present in adults)
*aka mucocutaneous lymph node syndrome
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
Kawasaki disease - important complication
*can develop coronary artery aneurysms
Kawasaki disease - treatment
*high dose salicylates (aspirin)
*IVIG
*echo to evaluate for aneurysms (initially + 2 weeks + 6-8 weeks)
ANCA vasculitis
*small vessel vasculitis
*p-ANCA = MPO
*c-ANCA = PR3
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
granulomatosis with polyangiitis (GPA) - diagnosis
*clinical history
*lung or kidney biopsy:
-granulomas in tissue
-pauci-immune glomerulonephritis
*c-ANCA, anti-PR3+
granulomatosis with polyangiitis (GPA) - treatment
*high dose steroids
*cyclophosphamide or rituximab
microscopic polyangiitis (MPA) - symptoms
*lower respiratory tract + kidneys (no upper airway involvement)
*fever, arthralgia, palpable purpura
*rapidly progressive glomerulonephritis (pauci-immune)
*pulmonary hemorrhage
microscopic polyangiitis (MPA) - diagnosis
*biopsy skin, lung, or kidney (necrotizing but not granulomas)
*p-ANCA, anti-MPO+
microscopic polyangiitis (MPA) - treatment
*high dose steroids, cyclophosphamide or rituximab
*relapse rate higher than GPA
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
eosinophilic granulomatosis with polyangiitis (EGPA) - diagnosis
*biopsy: granulomatous, necrotizing vasculitis with eosinophilic tissue involvement
*p-ANCA + (anti-MPO) (only in 40%)
*peripheral eosinophilia (>10%)
eosinophilic granulomatosis with polyangiitis (EGPA) - treatment
*high dose steroids
*maybe cyclophosphamide or rituximab if severe
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)
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)
IgA vasculitis - treatment
*supportive
*NSAIDs
*prednisone if severe renal involvement
Behcet’s syndrome
*a small vessel vasculitis
*common in Mediterranean descent
*oral and genital ulcerations
*other manifestations: uveitis, rash, pulmonary hemorrhage
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
type 2 cryoglobulinemic vasculitis - associated with…
hepatitis C
type 3 cryoglobulinemic vasculitis - associated with…
autoimmune disease
cryoglobulinemic vasculitis - symptoms
* purpura - lower extremity/dependent areas
*mononeuritis multiplex
*immune complex glomerulonephritis
cryoglobulinemic vasculitis - labs
*low complements (C4)
*Hep B, Hep C, HIV
*RF + but negative CCP
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
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
cocaine-induced vasculitis - labs
*p-ANCA positive
*MPO negative
*can be PR3 positive
*anti-human neutrophil elastase positive
*agranulocytosis
cocaine-induced vasculitis - treatment
*supportive
*stop cocaine
