Pathoma- Vasculitis Flashcards
What symptoms are common to all vasculidities?
- nonspecific symptoms (myalgia, malaise, etc)
2. symptoms of organ ischemia (depending on the organ)
What are 2 ways organ ischemia can occur?
- thrombosis in a BV
2. fibrosis of the BVs
What are the 2 large vessel vasculidities?
- temporal (Giant Cell) arteritis
2. Takayasu Arteritis
What is temporal (Giant Cell) arteritis?
the most common form of vasculitis in older adults, usually affects females over age 50; manifests in carotid artery and its branches
What are the s/s of temporal (Giant Cell) arteritis?
headache visual disturbances jaw claudication flu-like symptoms (joint and muscle pain) elevated ESR (>100)
What is seen in the biopsy of temporal (Giant Cell) arteritis?
inflamed vessel wall
giant cells (due to granulomatous vasculitis)
intimal fibrosis
segmental lesions
What is the tx for temporal (Giant Cell) arteritis?
corticosteroids ASAP
Why is tx so important in temporal (Giant Cell) arteritis?
high risk of blindness w/o tx
How are Takayasu Arteritis and temporal (Giant Cell) arteritis different?
in Takayasu:
- younger than age 50, usually young Asian females
- usu aortic arch at branch points
- weak or absent pulse in upper extremities
What BVs do the medium-vessel vasculidities supply?
they’re muscular arteries that supply organs
Name the medium vessel vasculidities.
- Polyarteritis Nodosa
- Kawasaki Disease
- Buerger Disease
What are the features of Polyarteritis Nodosa?
- multiple artery inflammation
- necrotizing vasculitis
- involves most organs EXCEPT THE LUNG
- in young adults
- serum HBsAg (hep B surface antigen)
How do the Polyarteritis Nodosa lesions look on imaging?
string-of-pearls nodulation (fibrinoid necrosis)
What is the tx for Polyarteritis Nodosa?
corticosteroids and cyclophosphamide
What happens in untreated Polyarteritis Nodosa?
death
Who does Kawasaki Disease affect?
Asian children <4yo
What is the presentation for Kawasaki Disease?
fever
conjunctivitis
erythematous rash of palms and soles
enlarged cervical lymph nodes
What artery is preferred in Kawasaki Disease?
the coronary artery
What is the tx for Kawasaki Disease?
aspirin and IVIG
What vessels does Buerger Disease affect?
BVs of the fingers
Buerger Disease is only associated with _____.
smokers
What is Raynaud phenomenon?
discoloration of the fingers/toes bc of vasospasm
What is the presentation for Buerger Disease?
- necrotizing vasculitis of digits (ulcers, gangrene, autoamputation)
- Raynaud phenomenon
What is the tx for Buerger Disease?
smoking cessation
What are the small vessel vasculidies?
- Wegener Granulomatosis
* WeCKener - Microscopic Polyangiitis
- Churg-Strauss Syndrome
- HSP
What does Wegener Granulomatosis look like?
- necrotizing granulomatous vasculities involving the nasopharynx, lungs, and kidneys
- serum C-ANCA
- sinusitis/nasopharyngeal ulceration
- hematuria due to rapidly progressive glomerulonephritis (RPGN)
What is the tx for Wegener Granulomatosis?
cyclophosphamide and corticosteroids
What is the ANCA test?
neutrophil on a slide is given pt’s serum- if Abs are present, they will present near the nucleus (P-ANCA) or on the outer rim of the nucleus (C-ANCA)
What are the features of Microscopic Polyangiitis?
- necrotizing vasculitis of multiple organs, esp lung and kidney
- P-ANCA
- NO granulomas or nasopharyngeal involvement
What is the tx for Microscopic Polyangiitis?
cyclophosphamide and corticosteroids
What is Churg-Strauss Syndrome?
- necrotizing granulomatous vascultis with eos and asthma
- multiple organs, esp lungs and heart
- P-ANCA
What does HSP stand for?
h___ine purpura
What is HSP?
- vasculitis due to IgA immune complex deposition
2. most common vasculitis of children
What is the classical presentation of HSP?
- classical purpura on buttocks and legs
- GI pain and bleeding
- hematuria (kidney IgA nephropathy)
- post URI/viral infection
