11. Vascular Pathology Flashcards
What is Polyarteritis nodosa (PAN)?
Segmental necrotizing vasculitis; it is a systemic vasculitis of small or medium-sized muscular arteries, typically involving renal and visceral vessels but sparing the pulmonary circulation. Symptoms result from ischemic damage to affected organs, often the skin, heart, kidneys, and nervous system. Generalised symptoms include fever, fatigue, weakness, loss of appetite, and weight loss. Muscle and joint aches are common.
What organ does polyarteritis nodosa avoid?
The lungs.
What are the three stages of damage that polyarteritis nodosa inflicts?
I. Acute lesion: fibrinoid necrosis and neutrophils. II. Healing lesions: fibroblast proliferation. III. Healed lesions: nodular fibrosis and loss internal elastic lamina.
What are possible complications of PAN?
Thrombosis, infarction, and aneurysms (kidney, heart, and GI tract).
What are some laboratory findings of PAN?
Perinuclear antineutrophil cytoplasmic autoantibodies (P-ANCA); they are autoantibodies against myeloperoxidase. It correlates with disease activity.
How is PAN diagnosed?
Arterial biopsy.
What is the treatment for PAN?
Corticosteroids and cyclophosphamide.
What is Churg-Strauss syndrome?
AKA allergic granulomatosis and angitis, it is a variant of polyarteritis nodosa, associated with bronchial asthma, systemic vasculitis with granulomas and eosinophilia, and involves the lung, splee, kidney, etc.
What is Wegener granulomatosis?
Necrotizing vasculitis with granulomas; classically involves the nose, sinuses, lungs, and kidneys. It affects small size arteries and veins.
What are clinical presentations of Wegener granulomatosis?
Bilateral pneumonitis with nodular and cavitary pulmonary infiltrates, chronic sinusitis, nasopharyngeal ulcerations, renal disease (Focal necrotizing glomerulonephritis, crescentic glomerulonephritis). Micro: fibrinoid necrosis, neutrophils, and granulomas.
What are some laboratory findings of Wegener granulomatosis?
Cytoplasmic antineutrophil cytoplasmic autoantibodies (C-ANCA); they are antibody against proteinase 3, correlates with disease activity. [Waggy Cunt]
What is the treatment for Wegener?
Immunosuppressive drugs (cyclophosphamide).
What is temporal arteritis?
Segmental granulomatous vasculitis of small and medium-seized arteries such as the cranial arteries (temporal, facial, and ophthalmic arteries), and rarely aortic arch (giant cell aortitis). Multinucleated giant cells and fragmentation of the internal elastic lamina and intimal fibrosis with luminal narrowing. AKA giant cell arteritis, it primarily affects the elderly population.
What are the symptoms of temporal arteritis?
Throbbing headache, tender and firm temporal arteries, visual disturbances (blurred or double vision, visual loss), facial pain, fever, malaise, weight loss, muscle aches, anemia. Polymyalgia rheumatica: systemic flu-like symptoms and joint involvement.
What are some laboratory findings of giant cell arteritis?
Elevated erythrocyte sedementation rate (ESR) and associated with HLA-DR4.
What is Takayasu arteritis?
Granulomatous vasculitis with massive intimal fibrosis, irregular fibrous thickening of the wall of the aortic arch, and narrowing of the orifices of the major arterial branches. Affects medium-sized to large arteries, aortic arch and major branches. AKA pulseless disease, affects young and middle-aged asian women.
What are the clinical presentations for Takayasu arteritis?
Loss of pulse in the upper extremeties, ocular manifestations (visual loss or field defect, retinal hemorrhages), and neurologic abnormalities.
What is Buerger disease?
Recurrent neutrophilic vasculitis with microabscesse, segmental thrombosis that leads to vascular insufficiency. Affects small and medium-sized arteries and veins; involves the extremeties.
What is Buerger disesase strongly associated with?
Smoking.
What are the clinical presentations for Buerger disease?
Severe pain (claudication) in the affected extremity, thrombophlebitis, Raynaud phenomenon, ulceration and gangrene.
What is the treatment for Buerger disease?
Smoking cessation.
What is mucocutaneous lymph node syndrome?
AKA Kawasaki disease, it is segmental necrotizing vasculitis, weakened vascular wall may undergo aneurysm formation. Affects all calibers of arteries (coronary artery commonly affected [70%]).
