Vasculitides Flashcards
What is the most common arteriitis in adults?
temporal (giant cell) arteritis
What is temporal (giant cell) arteritis?
What vessels does it typically effect and what changes occur in the vessel?
granulomatous, large vessel vasculitis
Vessels:
-carotid branches (cranial arteries) , particularly temporal artery
Changes:
- segmental granulomatous changes, multinucleated giant cells
- fibrosis
What is the clinical presentation of temporal (giant cell) arteritis?
Constitutional Sx:
- fever, malaise, weight loss
- headache (temporal A.; actually occurs along artery which may also be TTP)
- visual changes (ophthalmic A.); **sudden loss of vision**
- jaw claudication (facial A.)
What is the epidemiology of temporal (giant cell) arteritis?
- more common in females
- older adults (>50)
- european descent
What are diagnostic features of temporal (giant cell) arteritis?
-segmental granulomatous changes noted on temporal A. biopsy (because of segemental nature, false negative biopsy can occur)
-elevated ESR
What is the treatment and prognosis for temporal (giant cell) arteritis?
-corticosteroids upon ***suspicion of disease*** to prevent blindness (even before biopsy)
What are major complications of temporal (giant cell) arteritis?
- sudden onset, potentially permanent blindness; this is why you treat upon suspicion before biposy confirmation
- aortic aneurysm
- ischemic stroke
What other condition is often present with temporal (giant cell) arteritis?
-polymyalgia rheumatica (arthralgia and myalgia of shoulder and pelvic girdles)
What is Takayasu arteritis?
What vessels does it typically effect and what changes occur in the vessel?
granulomatous, large vessel vasculitis
Vessels:
-aortic arch at branching points (crainal and upper ext As.)
Changes:
- smooth tapered stenosis
- granulomatous changes of aortic arch, multinucleated giant cells
- fibrosis
What is the epidemiology of Takayasu arteritis?
- more common in females
- adolescents to mid adults (15-45)
- more common Asians (Takayasu is Japanese -> Asian)
“young asian women”
What is the clinical presentation of Takayasu arteritis?
Constitutional Sx:
- fever, malaise, weight loss
- decreased radial/brachial pulses (“pulseless disease”)
- vision changes; “copper-wiring” retinopathy
- HTN (renal A.)
What are diagnostic features of Takayasu arteritis?
- smooth stenosis on angiography
- elevated ESR
What is the treatment and prognosis for Takayasu arteritis?
- corticosteroids
- typically regresses but aquired permanent changes (vision and neuro deficits) will remain
What is a major differentiating factor between temporal arteritis and Takayasu arteritis?
age; Takayasu <50 and temporal >50
- very similar with constitutional s/x, visual changes, and neuro s/x
- very similar histology
What is polyarteritis nodosa?
What vessels does it typically effect and what changes occur in the vessel?
necrotizing systemic medium vessel arteritis
Vessels:
- muscular, medium arteries suppling most organs
- ***lungs spared***
Changes:
- transmural inflammation w/ fibrinoid necrosis (early) -> fibrous aneurysms (“string of pearls”; hence nodosa) (late)
- multiple, diffuse lesions (hence polyarteritis) found in varying stages
What is the clinical presentation of polyarteritis nodosa?
Constitutional Sx:
-fever, malaise, weight loss
Symptoms are manifestaions of organ ischemia** and **variable
- ulcers and gangrene
- HTN (renal A.)
- abdominal pain with melena (mesenteric As.)
- mononeuritis multiplex (foot drop)
What is the epidemiology of polyarteritis nodosa?
-more common in males
-young adults
What are diagnostic features of polyarteritis nodosa?
- multiple microaneurysms “string of pearls” on angiography
- fibrinoid necrosis on biopsy
-positive hepatitis B
What other condition is often present with polyarteritis nodosa?
hepatitis B infection; positive for HBsAg (hepaitits B surface Ag
What is the treatment and prognosis for polyarteritis nodosa?
- corticosteroids
- cyclophosphamide
**fatal if not treated**
What is Kawasaki disease?
What vessels does it typically effect and what changes occur in the vessel?
necrotizing medium vessel vasculitis
Vessels:
-muscular, medium arteries (of significance are coronary As.)
Changes:
- similar to PAN, less severe
- mostly insignificant other than in cornary As.
- transmural inflammation w/ fibrinoid necrosis -> aneurysms
- thrombosis (following vessel damage)
What is the clinical presentation of Kawasaki disease?
CRASH and BURN
- Conjunctivitis
- Rash (trunk)
- Adenopathy (cervical)
- “Strawberry” tongue (bright red and bumpy)
- Hands and feet (rash)
- burn -> fever
very similiar in appearance to a viral infection
What is the epidemiology of Kawasaki disease?
- children (<5)
- most common in Asians
What are diagnostic features of Kawasaki disease?
- fever >5 days + 4/5 CRASH s/x
- echocardiogram to assess coronary arteries
What is the treatment and prognosis for Kawasaki disease?
- aspirin to prevent thrombosis of coronary As. (**aspirin is not normally given to children, esepcially when febrile, so this is unusual**)
- IVIG
- self limited though can have lasting cardiac damage
- increased risk of MI
What is thromboangiitis obliterans (Buerger disease)?
What vessels does it typically effect and what changes occur in the vessel?
medium vessel vasculitis
Vessels:
-muscular, medium arteries (particularly those of the extremities)
Changes:
- smooth, tapering occlusion of vessels
- formation of “corkscrew” collaterals
- segemental thrombosis
What is the clinical presentation of thromboangiitis obliterans (Buerger disease)?
Early triad:
- Raynaud phenomenon
- superficial thrombophlebitis (migratory)
- claudication (cramping)
Late:
-ulceration, gangrene, and autoamputation of fingers/toes
What is the epidemiology of thromboangiitis obliterans (Buerger disease)?
-young men (<35)
***only seen in smokers***
What are diagnostic features of thromboangiitis obliterans (Buerger disease)?
- segmental, smooth tapering lesions of distal vessels of extremities
- “corkscrew” collaterals
What is the treatment and prognosis for thromboangiitis obliterans (Buerger disease)?
- no effective treatment
- smoking cessation stops sympsoms
- aquired damage will persist
What is granulomatosis with polyangitis (Wegener granulomatosis)?
What vessels does it typically effect and what changes occur in the vessel?
granulomatous and necrotizing small vessel vasculitis
Vessels:
-small vessels (of significance are upper resp, lower resp, and kidney)
Changes:
-lesion have central necrosis with peripheral granulomas
What is the clinical presentation of granulomatosis with polyangitis (Wegener granulomatosis)?
Triad of upper respiratory, lower respiratory, and renal
Upper respiratory:
- rhinitis/sinusitis
- ulcerations -> septal perforation
- saddle nose
Lower respiratory:
- hemoptysis
- nodular infiltrates -> cavitating lesions (pneumonia-like s/x)
Renal:
- rapidly progressive glomerulonephritis (crescentic)
- hematuria
What is the epidemiology of granulomatosis with polyangitis (Wegener granulomatosis)?
- more common in males
- older adults
What are diagnostic features of granulomatosis with polyangitis (Wegener granulomatosis)?
- biopsy shows necrotic, granulomatous vasculitis
- positive c-ANCA (autoantibody)
- nodular lesions on CXR
What is the treatment and prognosis for granulomatosis with polyangitis (Wegener granulomatosis)?
-corticosteroids
(reduce ANCA)
- cyclophosphamide
- methotrexate
- rituximab
- relapses are common
- lethal without treatment
What is eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?
What vessels does it typically effect and what changes occur in the vessel?
necrotizing, granulomatous small vessel vasculitis w/ eosinophilia
Vessels:
-small vessels throughout the body
Changes:
- necrosis and granuloma of vessels
- extravascular infiltration by eosinophils
What is the clinical presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?
Primarily affecs lungs and skin
- asthma
- palpable purpora
- GI bleeding
- glomerulonephritis
- pericarditis
What are diagnostic features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?
- eosinophila
- necrotizing granulomas
- pANCA (<50%)
What is the most common cause of death in Churg-Strauss syndrome?
-cardiac (cardiomyopathy, myocarditis, infarction)
What is Behcet syndrome?
What vessels does it typically effect and what changes occur in the vessel?
neutrophilic vasculitis of small to medium vessels
What is the clinical presentation of Behcet syndrome?
Tirad:
- oral ulcers
- genital ulcers
- uveitis (inflammation of the eye)
- GI ulcers
- neuro s/x (similar to MS)
- arthralgia
What is the epidemiology of Behcet syndrome?
Turkey, Asia, and the Middle East
What immunophenotype is associated with Behcet’s disease?
-HLA-B51
What is microscopic polyangiitis?
What vessels does it typically effect and what changes occur in the vessel?
necrotizing small vessel vasculitis
Vessels:
-capilaries and venules
Changes:
- transmural inflammation w/ fibrinoid necrosis -> segmental fibrous aneurysms (very similar to PAN)
- neutrophil infiltration
- diffusely uniform stage of disease activity (unlike PAN)
What is the clinical presentation of microscopic polyagniitis?
Kindey:
- pauci-immune (no immune complex) glomerulonephritis -> hematuria/proteinuria
- HTN
Lungs:
-hemoptysis
Skin:
-palpable purpora
Nerves:
-weakness
What are diagnostic features of microscopic polyangiits?
- fibrinoid necrosis on biopsy w/ no granulomas
- pANCA (but no immune complex deposition)
What vasculitides have a similar presentation to microscopic polyangiitis?
How are they differentiated?
**all the similar vasculitides can affect medium vessels while microscopic polyangiitis cannot
PAN:
-lesions in varying stages
Wegner’s:
-granulomas
Churg-Straus:
-eosinophilia
What is Raynaud’s phenomenon?
vasospasm of small arteires of fingers and toes
can be primary or secondary
What are precipitating factors for Raynaud’s phenomenon?
- cold
- stress
What features differntiate primary and secondary Raynaud’s?
Primary:
- more common in females
- symmetric
- episodic, can have remission
Secondary:
- associated with other conditions such as SLE, scleroderma, or thromboagniitis obliterans
- asymmetric
- progressive
