Vasculitis Flashcards
What is vasculitis?
inflammation of blood vessels w/ necrosis of blood vessel wall, narrowing, and occlusion
what are the major signs and symptoms for vasculitis?
- fever, myalgia, athralgia, malaise
- palpable purpura, focal skin necrosis, livedo reticularis, urticartria, myositis, etc
What happens in livedo reticularis?
blood vessels are dilated
How are the different types of vasculitis classified?
size of blood vessel involved
What are the large vessel vasculitis?
Giant cell temporal arteritis and Takayasu Arteritis - both granulomatous, Giant cell is older people while Takayasu is less than 50 yr olds
What are the medial vessel vasculitis?
- PAN
- Kawakasi Dz
- Buerger
- Cutaneous PAN
What are the small vessel vasulitis?
- Wegners - GPA, lung, kidney, and URT ( C-ANCA)
- Churg Strauss - eosinophils and asthma (p-ANCA)
- MPA - no URT but yes to lung and kidney (pANCA)
- HSP - skin, gut, and kidney – IgA
- Essential Cryoglobulinemia - skin and kidney
- Leukocytoclastic vasculitis - HSR vasculitis
What vasculitis have granulomas?
- giant cell
- takayasu
- cogan
- wegners
- churg strauss
- Buerger
- Rheumatoid vasculitis
What is PAN?
- necrotizing, medium
- spares small vessels
- no GN or granuloma
- MEN
- Hep B
What vasculitis have GN?
- MPA
- HSP
- Wegner
- Churg Strauss
What are the clinical manifestations of PAN?
- systemic symptoms
- peripheral neuropathy
- HTN and renal failure
- GI problems -
- testicular involvement
- cadiac involvement
- skin — LIVEDO RETICULARIS
- SPARES LUNG!
What is the histology of PAN?
Fibrinoid Necrosis, thrombosis, acute inflammatory cell exudates, aneurysm formation
What will be see in lab tests for PAN?
- A, T, E, C
- hematuria
- elevated creatinine
what happens in the acute phase and late phase of PAN on histo?
- acute - PMN and fibrinoid necrosis
2. late - chronic inflammation, fibroblasts, and scarring
What phase are all the lesions in PAN on histo?
different phases – pearl on a string appearance!
What is cutaneous PAN?
PAN that is limited to the skin, associated w/ Hep C. Myalgia and peripheral neuropathy seen.
What is Buerger’s dz? (thomboangitis obliterans)
- affects the vascular supply to the lower limbs in young adult male tobacco smokers - South Asians
- Abs including ANCAS against MPO have been associated w/ sever dz
What symptoms do Buerger’s pts have?
begins w/ bilateral pain and ischemia in lower extermities, pain on exposure to cold may exist
How does one stop Beurger’s?
STOP SMOKING - helps with the rapid evolution of the disease to ischemia
What signs can be presents for Beurger’s?
limb pain, digital cyanosis, splinter hemorrhages, and skin/digital ulcers
What is seen for Buerger’s for histo?
segmental, transmural w/out necrosis; Granuloma present
What is Cogan’s syndrome?
- rare, young adults w/ red/painful eyes and/or hearing loss within 4 months
- rarely you get widespread vasculitis w/ purpura and gangrene
What is Susac’s syndrome?
- rare, endotheliopathy, wide age range
- sudden onset of sensorineural hearing loss w/ encelphalopathy and branch retinal artery occlusion
- DDx includes - Cogan’s syndrom and GPA
What is Microscopic Polyangitis? MPA
- necrotizing - small vessels
- GN
- p-ANCA
- more common than PAN
What are the clinical manifestations of MPA?
- systemic symptoms
- GN
- lung - pulmonary hemorrhage
- neuropathy
- skin
Lab findings for MPA
- A, T, E, C
- RBC casts
- elevated creatinine
- p-ANCA
What are the most common type of IC-mediated vasculitis?
- HSR vasculitis
- HSP
- mixed cryoglobulinemia
What is the most prominent feature of IC-mediated vasculitis?
cutaneous involvement of small blood vessels
What is the classic cutaneous finding in small vessel vasculitis?
palpable purpura
What is HSP associated w/ in terms of symptoms/signs?
- purpura
- arthritis
- GN
- colicky ab pain
What is cryoglobulinemic vasculitis most often associated w/?
long standing Hep C. Also you have both IgM and IgG
What is HSR vasculitis?
small vessel vasculitis of skin that spares internal organs and usually following drug exposures – withdraw drugs in adults
What is HSP?
necrotizing vasculitis of small vessels. Most common in children after an URT infection and usually resolves. IgA deposition!
What is seen on histo for HSP in early and late stages?
- neutrophils w/ breakdown - dispersing nuclear dust
- later - lymphocytic infiltration
– lesions will be in the same stage due b/c of immune mediated by exposure
Lab for HSP
- A, T, C, E
2. hematuria, elevated creatinine, RBC casts if renal involvement
What is cryoglobulinemia vasculitis?
cryoglobulins that preciptate from serum under conditions of cold and resolubilize upon rewarming.
Associated w/ Hep C
What are the different type of cryoglobulins?
Type 1: monoclonal, RF negative, associated w/ malignancy - cause is hyperviscosity syndrome not vasculitis
Type 2: polyclonal IgG, monoclonal IgM, RF positive, IC mediated vasculitis is the cause
Type 3: polyclonal, RF positive, IC mediated vasculitis is the casue
What are the clinical presentations of Cryoglobulinemic vasculitis?
- palpable purpura w/ ulcers
- peripheral neuropathy
- Raynaud’s
- Digital ischemia
- Secondary Sjogrens
What are the lab findings for Cryoglobulinemic?
- A, T, E,
2. low C4 levels, RF positive
Rheumatoid vasculitis
- medium and small blood vessels
- deep cutaneous ulcer over malleoli are hallmark of RV
- biopsy shows granular IgM and C3 deposition in vessels.
What organs does GPA usually affect?
- upper and lower RT
- kidneys
- lungs
- pts can get saddle nose deformity, ulcerations
What is the antibody in GPA?
c-ANCA for proteinase 3 antigen
What is AGPA?
prior history of adult onset asthma or allergic rhinitis and tissue eosinophilia w/ necrotizing vasculitis and extravascular granuloma formation
What is the pathology of GPA?
necrotizing granulomatosis, geographic area of necrosis w/ basophilic debris and palisaded histocytes – like Rheumatoid nodule
What antibody can be found in AGPA?
pANCA
What are the clinical manifestations of AGPA?
- systemic symptoms
- lungs - dyspnea and cough
- peripheral neuropathy
- GU involvement
What is giant cell arteritis?
- large sized - necrotizing inflammation
- granuloma
- AM stiffness, pain in pelvic girdle, shoulders
- old white women
What are the clinical presentations of giant cell arteritis?
- temporal headaches
- visual disturbances
- jaw/tongue claudication
- tender, swollen, temporal artery
Pathology of giant cell arteritis?
- segemental, transmural
- lymphocytes that affects internal or external elastic membrane,
- granuloma w/ multinucleated giant cells
- inflammation is most marked near elastic membrane
What is crucial about giant cell in terms of Rx?
give corticosteroids to prevent the blindness, don’t even wait for biopsy just give them meds!
What is Takaysu’s arteritis?
necrotizing, large vessels (aortic arch and its branches affects), granuloma. Young Asians
Clinical manifestations of Takaysu’s?
- renal - renovascular HTN
- GI - N/V/IBD
- cardiac
- CNS
- Muscoskeletal symptoms
What is seen on pathology of Takaysu’s?
- segmental, necrotizing, lymphocytes
- intimal and adventital thickening
- medial loss of elastic fibers = thinning = perforation likely
What is Kawaski?
necrotizing, coronary arteries – give Aspiring and IvIg
Clinical manifestation of Kawaksi?
- Rash
- bilateral conjunctival injection
- cervical LAD
- extremity changes
- mucus membrane changes - strawberry tongue
Pathology for Kawaksi?
endothelial necrosis, transmural w/ PMN, lymphocytes, wall necrosis, Aneurysm, thrombosis — MEDIA Thickened
What is primary angitis of CNS?
- vasculitis confined to brain, meninges, or spinal cord
- dx based on spinal fluid, brain/vascular imaging, biopsy
- many subsets exists such as granulomatosis PACNS
What is GPACNS
- affects middle aged med, present w/ chronic insidious headaches along w/ diffuse and focal neurologic deficits
- commonly have infarcts, high T2 mediated fluid attentuation on MRI
- dont’ do a cerebral angiogram
What is Behcet’s dz?
- complex multisystem - oral and genital ulcers
- cutaneous lesions - PMN vascular
- highest prevalence in silk route!
- HLA B51, think heat shock proteins
What is the Dx of Behcet’s based on?
oral aphthae, genital aphthae, ocular lesions, cutaneous lesions, and a positive pathergy test
