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34 Cards in this Set
- Front
- Back
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What branches of arteries does GCA affecte? |
2nd to fifth branches of the aorta, including the external carotid,s temporal arteries, ciliaryh and opthalmic arteries; can affect the subclavian, uncommonly intracranial arteritis can occur |
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What is the pathogenesis of GCA? |
Dendritic cells in the vessel adventita become activated anr ecruit T cells and monocytes into the vessel wall, macrophages then coalesce into multinucleated giant cells which secrete metalloproteinases, , intimal proliferation also can cause reduced blood flow and partial/complete ischemia of the affecte tissue bed |
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What are the clinical manifestations? |
Scalp pain, headache, or tenderness over the temporal artery, which is usually unilateral - constitutional symptoms - pain in the msucles of mastication during chewing - most feared outcome is blindness, caused by opthalmic or posterior ciliary artery occlusion - can be preceeded by amaurosis fgax - occ can involve the brachial arteries and cause decreased pulses and forearm ischemia; aortic anuerysm is a potential complication - Definitely diagnosed by biopsy |
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What is teh treatment of GCA? |
- Do not delay treatment for biopsy results because treatment is highly effective and prevents blindnes,s and biopsy specimens remain interpretable for at least 2 weeks after treatment initiation - 60mg/d prednisone or 1mg/kg/d, in patient s/w visual loss, can do IV steroids (1g solumedrol TID), w/10 % reduction every few weeks - Treatment duration can last 6-18 months, low dose aspirin daily can fruther reduce the risk of blindness - Use ESR as a marker of disease activity |
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What are the clinical manifestations of PMR? |
= 30-50% of patients w/ GCA have PMR, 10-15% of patients with PMNR but no clinical GCA have occlut GCA on blind temporal artery biopsy - Symmetric pain and stiffness of muslces in the shoulder, neck and hips, without actual joint arthritis - Inability to raise from a chair or comb hair - Can be associated with synovitis |
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What is the treatment? |
- low dose prednisone 10-2omg/d, most patients respond w/in 3 days - Frequent partial relapses during prednisone tapering, some take years to taper completely off pred, others continue on it indefinitely |
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What is the presentation of takayasu's arterities? |
= Aorta: ascending, descending, abdominal aorta and majro branches; TA is very rare compared to GCA, and primarily affects young women w/ typical onset between 15 to 25 years - Constitutional symptoms, maylgias/arthralgias - Diminished or absent pulses superficially, bruits, BP differences on both sides - Can have HTN if there is coarctation resulting in decreased kiendy perfusion |
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How is takayasu's diagnosed? |
- Angiogram demonstrates focal arterial narrowing and/or aneurysms, lab testing is nonspecific but typically reveals an elevated ESR |
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How is TA treated? |
- Untreated conveys early morbidity and mortality - High dose pred 1mg/kg followed by tpaer - Patients w/ structural damage ot affected vessels can require angioplasty, bypass or reconstruction |
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What is the epi of PAN? |
-30% of cases are associated with HBV, vaccination reduces PNA incidence |
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What vessels do PAN affect? |
- medium and small vessels, causes vessel narrowing anuerysms and microaneurysms; classic features of PAN result from ischemia and/or aneurysm rupture - Kidneys, GI tract (mesenteric artery and small intestine), peripheral nervous system and skin |
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What are the clinical manifestations of PAN? |
Constituional systems, HTN from decreased perfusion to kidney, chronic or intermittent ischemic pain (esp after eating), resulting in bowel infarction - Neurological_ mononeuritis multiplexu - Skin: livideo reticularis, purpura, painful subcutaneous nodules |
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How is PAN diagnosed? |
- Elevated inflammatory markers, HBV antigenemia - Vascular imaging studies: mesenteric/renala rtery imaging with angiography or CT angiography reveals medium sized artery aneurysms or stneosis |
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What is the treatment of PAN? |
- High dose prenidsone and cylcophosphamide; methotrexate, azathioprine, cellcept can be sued as alternatives for mild disease or for maintenacne therapy |
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What are the manifestations of PACNS? |
Very rare 2.4 cass/million - Median age is 50 years, man >> women, vascular involvement is limited to intracerebral vessels, necrotizing granulmatous vasculitis is typical |
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What are the manifestations of PACNS? |
-Recurrent HAs, progressive encephalopathy - Strokes, TIAs, visual field defects, seizures can occur; no systemic involvement and systmeic inflammatory markers are not elevated |
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How is PACNS diagnosed? |
LP, MRI, cerebral angiography and brain biopsy - CSF reveals elevate dprotein, lymph predominant lymphocytosis - MRI shows multiple diffuse and focal abnormalities, nonspecific - IC angiography: ectasia and stenosis - Defnitive test is brain biopsy which shows granulomatous vasculitis |
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How is PACNS treated? |
High dose steroids and cyclophosphamide |
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What are the ANCA associated small vessel vasculitises? |
Wegeners (polyangitis with granulomatosis), microscopic polyangitis, eosinophilic granulomatosis w/ polyantigis (church strauss) |
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What is p-ANCA, c-ANCA? |
p-ANCA is perinuclear anti-neutrophil antibody directed toward neutorphil enzyme myeyloperoxidase (MPO) c-ANCA (diffuse staining across the cytoplasm) toward neutrophil proteinase 3 (PR3) |
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What is the epi of granulomatosis with polyangitis? |
- Most common ANCA associated vasculitis - TYpically affectes middle aged to older patients, white,northern europenams - 80-90% of patients have c-ANCA and anti-pR3 activity |
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What is the clinical presentation of granulomatosis w/ polyangitis? |
- Upper respiratory tract (sinuses, ears) lungs, kindeys - Nasal/sinus pain, congestion, rhinitis, epitaxis - Nasal inflammation can cause cartilage damage and collapse (saddle nose deformity), hearing loss can occur d/t eustachian tube damage - Damage to trachea: airway narrowing and obstruction - Ocular inflammation: blindness - Lungs: can have nodules and infitlrates, capillaritis can result in alveolar hemorrhage and hemoptysis - Kidneys: RPCG, - Skin: painful cutaneous nodules, palpable purpura, urticarial and ulcerative lesios and peripheral nerves |
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How is granulomatosis w/ polyangitis diagnosed? |
Tissue biopsy is usually necessary - c-ANCA positive - Granulomas are usually not present ins renal biopsy so it cannot distinguish GAP from other ANCA condtiions - Skin biopsies are also usually notndiagnostic - Lung biopsy is the best: most invasive but provides necrotizing granulomas (typical of GPA but not other ANCA diseases) |
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How is GPA treated? |
High dose steroids, cyclophosphamide for approximately 6 months, followed by maintenance w/ methotrexate, azathioprine or cellcept - Steroids alone are insufficient; recent studies suggest anti B cell antibody - rituxan as efficacious as cyclophosphamide, possibly w/ less toxicity - For limited disease, methtrexate and steroids can be sufficient - Using immunosuppreses, GPA morbidity has declined from 90% to 10% |
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What is the epi of MPA? |
- Shares features w/ GPA but differs in organ invovlement and autoimmunity - Half as frequent as GPA - Men > women - pANCA and anti-MPO |
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What is the organ involvement of MPA? |
vasculitis of the kidneys and lungs; cresent formation - biopsy will not show immune deposition - lung involvement occurs in half of the cases, consisting of nongranulomatous alveolar infiltrates that may be fleeting or persistent and are histologically reflected as pulmonary capillaritis w/ neutrophilic infiltrates; - DAH - Skin (purpura) is common |
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What is the treatment of MPA? |
Same as for GPA |
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What is the epi of EGPA? What are the clinical manifestations? |
Rare, strong association with astham suggests atopic trigger - Atopy, allergic rhinitis, nasal polyps, asthma - Lung disease w/ infiltrates, capillaritis - Peripheral nerve disease is more common in EGPA than in other ANCA vasculitides, presenting as mono or polyneuropathy or mononeuritis multiplex - Kidney disease less common - Hypereosinophilia - Tissue biopsy can show necrotizing vasulitis w/ eosinophilic infiltrates |
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How is EGPA diagnosed? Treated? |
- 40% are ANCA negative, neg ANCA should no t eliminate the diagnosis - p-ANCA, anti-MPO pattern - Treat w/ steroids in mild disease, steroids plus cyclophosphamide in serious diseases |
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What are the immune complex mediated vasculitis? |
- Immune complexes can deposit in small vessels, activate complement and recruit neutrophils - Any organ can be affected, but skin and joint involvement is common - Plapable purpur, worse in dependent areas,influxed neutrophils undergo cell death and breakup and release of pyknotic nuclei (leukocytoclastic vasculitis) |
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What are the different ytpes of cryoglobulins? |
Type I si a monoclonal antibody reuslting from hematologic malignancy - Type I rarely form immune complexes but can cause hyperviscosity - Type II consists of a polyclonal IgG antibody together with a monoclonal IgM with rheumatoid factor activity (binds to other immunoglobulins) contributing to immune complex formation - 90% of patients with type II cryo have chornic HCV cirus infection, which furnishes the antigen for immune complex formation - Remaining 10% are idiopathic and designated essential mixed cryoglobulinemia -Type III cyroglobulins are also mixed but consist of polyclonal IgG together w/ polyclonal IgM RF, occur typically with other autoimjune disease |
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What is the presentation of cyroglobulins? |
- Palpable purpura in type II cryoglobulinemia; other organs include peripheral nerves and kidneys - Less commonly, CNS, GI and other organ invovlement occurs |
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How is cryoglobulinemia diagnosed? |
Cryoglobulinemia - can be assessd directly in serum from blood that has been allowed to clot; the cryoglobulins when incubated at 4 degrees C forms cryoprecipitates - Negative RF argues against type II or III cyroglobulinemia |
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What is the management of cryoglobuliemia? |
- Treat the underlying cause - However, if involvement is severe, may require steroids, cyclophosphamide, plasmapheresis - more recently, anti-B cell therapy with rituxan has demonstrated efficacy at reducing cryoglobulinemia and can provide a less toxic alternative |
