04.06 and 04.07 - Vascular Disease, Aneurysms, Dissections, Vasculitis, etc. Flashcards Preview

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What is this?

Aneurysm cut open showing thrombus


What is the pathology of Kawasaki disease?

Endo necrosis + transmural inflammation with neutros, lymphos wall necrosis, aneurysms, and thrombosis


What do you see on the left and right?

- Left: interface of arterial intima and thrombosis

- Right: mixed acute and chronic arteritis


How do you treat Kawasaki disease? What is the prognosis?

- Treatment: aspirin and IVIg 

1. ASA risks Reye syndrome, but Kawasaki tips risk/benefit ratio in favor of giving it

- Prognosis: good (majority of aneurysms resolve); reduces risk of symptomatic coronary artery disease

- NOTE: Reye's rare but serious condition that causes swelling in liver and brain -> most often kids, teens recovering from viral infection like flu or chickenpox. Signs, symptoms of confusion, seizures, LOC need emergency tx -> early dx, tx can be lifesaving 


What is microscopic polyangiitis? What are its major clinical features?

- Aka, hypersensitivity or leukocytoclastic vasculitis: a necrotizing vasculitis that generally affects capillaries, small arterioles, and venules 

- All lesions tend to be of same age in pt

- Skin, mucous membranes, lungs, brain, heart, gut, kidneys, and muscle can all be involved

- Necrotizing glomerulonephritis (90% of pts) and pulmonary capillaritis are common

- Major clinical features: hemoptysis, hematuria, and proteinuria, bowel pain or bleeding, muscle pain or weakness, and palpable purpura

1. Except in those w/widespread renal or brain involvement, immunosuppression induces remission, markedly improves long-term survival


What is the pathogenesis of microscopic polyangiitis?

- Ab responses to Ags like drugs (penicillin), micro-organisms (streptococci), heterologous proteins, or tumor proteins -> can lead to immune complex depo or secondary immune responses (e.g., anti-neutrophil cytoplasmic antibodies, ANCAs) that are pathogenic 

- Most assoc w/anti-myeloperoxidase, MPO-ANCA (perinuclear, or P-ANCA)

- Recruitment, activation of neutros in vascular beds responsible for disease manifestations

- Segmental fibrinoid necrosis and in some areas (typically postcapillary venules), only infiltrating neutrophils, many undergoing apoptosis, are seen

- While Ig's, complement components can be in early skin lesions, little or no Ig's found in most lesions

1. Pauci-immune injury: vasculitis with minimal evidence of hypersensitivity on immuno-fluorescence and lack of Anti-glomerular BM Ab


What is granulomatosis with polyangiitis (Wegener's)?

- Uncommon, but typically whites in 40's 

- Necrotizing granulomatous vasculitis of both aa and vv in upper and lower respiratory tracts, kidneys 

- Geographic areas of necrosis with basophilic debris and palisaded histiocytes around them (like rheumatoid nodules -> see front of card) 

- 95% have anti-neutrophil cytoplasmic auto-Abs for proteinase-3 (ANCA-PR3; diffuse cytoplasmic, C-ANCA)


What is going on here?

- Mucosal ulcers 

- Necrotizing lesions 

- Can be destructive of nasal cartilage, leading to saddle nose deformity 

- Characteristic of: granulomatosis with polyangiitis 


What do you see here? Cause?

- Necrotizing, nodular lesions in the lung (become cavitating when largest and most advanced) 

- Characteristic of: granulomatosis with polyangiitis 


What is this?

- Geographic necrosis in granulomatosis with polyangiitis 


What do you see here?

- Multinucleated giant cells -> granulomatosis with polyangiitis 


What is this?

- Lung vasculitis 

- Granulomatosis with polyangiitis (affects both arteries and veins) 


What is going on here?

- Kidney with very small, tan nodular lesions in the medulla 

- Granulomatosis with polyangiitis 


What is going on in this interlobar artery in the kidney?

- Severe necrotizing vasculitis (low power trichrome stain) 

- Granulomatosis with polyangiitis 


What are the treatment and prognosis for granulomatosis with polyangiitis?

- Treatment: corticosteroids + either cytotoxic immunosuppressive therapy OR targeted therapy with Rituximab (anti-CD20)

- Prognosis: variable


What is allergic granulomatosis with polyangiitis?

- Aka, Churg Strauss syndrome

- Triad of asthma, eosinophilia, and vasculitis 

- Pathology: granulomatosis with polyangiitis + lots of eosinophils (simplified version)

- Image: necrotizing vasculitis 


What is going on here?

- Allergic granulomatosis with polyangiitis (Churg Strauss) 

- Numerous eosinophils recognizable by their red cytoplasma and bilobed nuclei (high power image of necrotizing vasculitis) 


What is this from? Describe the disease and treatment.

- Buerger Disease (thrombangiitis obliterans)

- Uncommon; young male Middle Eastern or South Asian smokers 

- Inflammatory, thrombosing vaso-occlusive disease of arteries and veins of limbs 

- Typically present with toe pain (+/- fingers), followed by ischemic ulcers, then gangrene -> distal to proximal 

- Pathology

1. Acute - segmental, transmural acute inflam w/o necrosis + thromboses + granulomas + giant cells 

2. Chronic - non-specific organization and recanalization of thrombus + neovascularization + fibrosis 

- Tx: stop smoking and amputate gangrene 


What do you see here? What might have caused this?

- Necrotic muscle around a thrombosed vein 

- Buerger disease (thromboangiitis obliterans) 


What do you see here? Just describe what this is.

- Chronic inflammation around an organized thrombus



What is peripheral arterial disease (PAD)? What are the signs and symptoms?

- Chronic atherosclerotic arterial disease of large and medium arteries, primarily of the legs 

- Common, primarily in the elderly (more comm in M)

- Symptoms: intermittent claudication (limb pain brought on by exercise and relieved by rest) 

1. Calf mm when disease in femoral, popliteal aa

2. Severe disease: pain at rest, ulcers 

- Signs: diminished/lost pulses, bruits (swishing sounds auscultated over lesions w/turbulent flow), pallor (or cyanosis), skin or muscle atrophy, ulceration, necrosis (gangrene)


What is this? How do you dx and treat this disease?

- Debrided leg ulcer from peripheral arterial disease (PAD)

Diagnosis: history and physical exam 

Treatment: exercise, esp. walking (develops collateral circulation) 


Tell me everything about acute arterial occlusion.

- Uncommon 

- Usually due to thromboemboli

1. 80% from heart: 65% mural from MI, 10% left atrial (atrial fibrillation, mitral stenosis), 5% dilated cardiomyopathy

2. 20% unidentifiable/miscellaneous sources 

- Signs and symptoms: FIVE P's -> paralysis, pallor, pain, paresthesia, and pulselessness in legs (70%) or arms (8%)

1. 10% are in the brain (o/sites include intestines, kidneys, and spleen)

- Diagnosis: history & physical examination

- Treatment: anticoagulation (heparin), intra-arterial thrombolytic therapy, catheter-based thrombectomy, surgery (thrombectomy, bypass)

- NOTE: acute arterial occlusion is often a SURGICAL EMERGENCY