Flashcards in Vasculitis Deck (49)
What are some symptoms common to vasculitis?
- palpable purpura (non-blanching)
- livedo reticularis (due to dilation of blood vessels)
VERY variable however
What would a blanching lesion indicate?
blood vessel swelling because you can physically push the blood out
What are the two major cause of vasculitis?
- immune mediated inflammation (most)
- direction invasion of vascular walls by infectious pathogens
What else can infections do?
cause a noninfectious vasculitis, by generating immune complexes or triggering a cross-reactive immune response
Immunologic injury in non-infectious vasculitis may be caused by:
- immune complex deposition
- anti-neutrophil antibodies (ANCA)
- antiendothelial cell antibodies
- autoreactive T cells
What are some vasculitis' that affect large vessels (aorta and its primary branches)?
- temporal (giant cell) arteritis
- Takayasu arteritis
What are some vasculitis' that affect medium vessels (muscular arteries and supply organs)?
- Polyarteritis nodosa
- Kawasaki Disease
- Buerger disease
What are some vasculitis' that affect small vessels (arterioles, capillaries, and venules)?
- Wegener's Granulomatosis
- Mircoscopic polynagiitis
- Churg-strauss syndrome
- Behcet disease
- Henoch-Scholein purpura
How do temporal arteritis (TA) patients present?
- older (50+), white females
- jaw pain when they chew
- ESR often elevated over 100
What is the etiology of TA?
T cell mediated
What is the treatment for TA?
What is the major complication of TA?
blindness- there is patchy ophthalmic artery involvement. Thus, treatment is started before diagnosis usually
What happens in TA?
- intima thickening, - inflammation of ALL 3 layers of the vessel
Half of patients with TA also have what?
What is polymyalgia rheumatica?
- proximal muscle aches and stiffness
- elevated ESR
- rapid resolution of symptoms with low-dose corticosteroid therapy
What is Takayasu arteritis?
granulomatous vasculitis (like TA- hist looks exactly the same) that classical involves the aortic arch at branch points and is common adults LESS than 50 y/o (primarily young, female Asians)
How do Takayasu arteritis patients present?
-younger than 50 yo
-visual and neurologic symptoms with weak/absent upper extremity pulse
Treatment for Takayasu arteritis?
-prognosis isn't that good
How does Polyarteritis nods (PAN) present?
-systemic inflammation, not localized
What organ is especially targeted by PAN?
renal involvement is the major cause of death
PAN is fatal unless treated with immunosuppression
PAN is associated with what?
strong association with hepatitis B
How can you differentiate PAN from Wegener's?
in PAN, the lungs are essentially never affected
How does PAN appear histo wise?
-segmental transmural necrotizing inflammation of small to medium sized arteries (can lead to aneurysm)
-fibrinoid necrosis is common
How does Kawasaki disease present?
-affects mostly Asian kids
-CRASH and burn:
Hand or feet changes/swelling/desquamation,
burn (fever 5+ days)
What does untreated Kawasaki disease lead to?
coronary aneurysm and MI
Treatment for Kawasaki disease?
-Aspirin (the only time you give aspirin)
Mircoscopically Kawasaki disease resembles what?
What is thromboangiitis Obliterates (Buerger Disease)?
a disease of younger smokers that causes inflammation and thrombosis of the arteries supplying the hands and feet, leading to claudication, cold sensitivity, ischemic pain, and eventually gangrene with autoamputation of digits
What is the treatment for Buerger disease?
cessation of smoking
What do the thrombi in Buerger disease contain?
small micro abscesses composed of neutrophils surrounded by granulomatous inflammation
the thrombus may eventually organize and recanalize
What is Raynaud phenomenon?
inflammation and vasoconstriction in response to cold or stress affecting the hands and feet leading to white digits (lack of blood flow), processing to blue color (cyanosis) and finally red (reactive hyperemia when blood flow is restored). Common in young women
How is Raynaud's phenomenon treated?
calcium channel blockers and avoiding cold
How does Granulomatosis with Polyangiitis (Wegener's) present? Triad?
- affects children and adolescents mostly
triad: granulomas, vasculitis, glomerulonephritis
What is a marker of GP?
c-ANCA (PR3-ANCA)- likely directly activates neutrophils to release oxygen radicals and proteolytic enzymes causing endothelial damage
How is GP treated?
cyclophosphamide and corticosteroids
What are some classical clinical findings of GP?
-chronic sinusitis with nasal petal perforation (saddle nose deformity), nosebleeds
- hemoptysis from lung involvement
- hematuria from renal involvement
How does microscopic polyangiitis (MP) present microscopically?
similar to Wegener with lung and kidney involvement but there are no granulomas
lesions are all the same age, unlike PAN
What antibody is MP associated with?
What is Churg Strauss Syndrome?
affects those with allergies/asthma (aka allergic granulomatosis and agnates)
CSS is associated with what?
What is Behcet disease?
clinical triad of recurrent oral aphthous ulcers, genital ulcers, and uveitis
How is Behcet treated?
immunosuppression with steroids or TNF-anatognists
What is the triad for Henoch-Schonlein Purpura?
-palpable purpura on legs and butt from inflamed vessels
-abdominal pain and blood stool
can lead to intussusception because swelling can act as a lead point to drag itself into the adjacent loop of bowel
common in kids
What does HSP follow usually?
viral upper respiratory infection
What causes HSP?
IgA immune complex deposition in mesangium capillaries (more benign than IgA nephropathy)
What is cryoglobulemia?
affects adults with hep C and these are proteins that precipitate in cold but redissolve with warming that when deposited causing inflammation and damage
What is infectious vasculitis?
direct invasion of infectious agents into vessel walls or can arise from hematogenous spread of microorganisms during septicemia or embolization from infective endocarditis
What agents commonly cause infectious vasculitis?
-usually bacteria (Pseudomonas))
-or fungi (Aspergillus and Mucor species)- common in diabetics