Chapter 7 ( Vascular ) Flashcards
Most common form of vasculitis in older adults (>50y) ?
Temporal ( Giant cell ) arteritis
Mechanism of Temporal arteritis ? Presentation ? Microscopic findings ?
Mechanism : Granulomatous vasculitis involving branches of Carotid artery
Presentation :
Headache ( temporal artery )
Visual disturbances ( ophthalmic artery )
Jaw claudication
Polymyalgia Rheumatica ( flue like symptoms with joint and muscle pain )
ESR is elevated
Microscopically : inflamed vessel wall , giant cells , intimal fibrosis , lesions are segmental ( requires biopsy of a long segment of the vessel )
Takayasu Arteritis mechanism ? Presentation ?
Granulomatous vasculitis involving the aortic arch at branch points
Presentation : Adults ( young asian female ) < 50 Visual and neurological symptoms Weak or absent pulse in the upper limbs ESR is elevated
Polyarteritis Nodosa mechanism ? Presentation ? Microscopic findings ? Ttt ?
Mechanism : necrotizing vasculitis involving multiple organs ( EXCEPT LUNGS )
Presentation :
In young adults
Hypertension ( renal artery )
Abdominal pain and melena ( mesentric artery )
Neurologic disturbances and skin lesions
Serum HBsAg
Microscopically :
Early , transmural inflammation with fibrinoid necrosis
Late , fibrosis producing String of Pearls appearance on imaging
Ttt : corticosteroids , cyclophosphamide , fatal if not ttt
Kawasaki Disease presentation ? Ttt ?
Presentation :
Asian children < 4 y
Non specific signs : fever , conjunctivitis , erythematous rash of palms and soles , enlarged cervical LN
Coronary artery involvement leads to thrombosis with MI , aneurysm with rupture
Ttt : ASPIRIN , IVIG
Wegener Granulomatosis mechanism ? Presentation ? Microscopically ? Ttt ?
Necrotizing granulomatous vasculitis involving nasopharynx , lungs , kidneys
Presentation :
Middle aged male
Sinusitis or nasopharyngeal ulceration
Hemoptysis with bilateral nodular lung infiltrates
Hematuria ( rapidly progressive glomerulonephritis )
Serum c-ANCA correlate with disease activity
Microscopically : large necrotizing granulomas with adjacent necrotizing vasculitis
Ttt : cyclophosphamide and steroids
Microscopic polyangiitis mechanism ? Presentaion ?
Necrotizing vasculitis involving multiple organs especially lungs and kidneys
Presentation : similar to Wegener Granulomatosis but NO NASOPHARYNGEAL INVOLVEMENT AND NO GRANULOMAS
Serum p-ANCA correlates with disease
Churg-Strauss syndrome mechanism ? Presentation ?
Necrotizing granulomatous inflammation with EOSINOPHILS involving multiple organs especially lungs and heart
Presentation :
Asthma
Peripheral eosinophilia
Serum p-ANCA correlates with disease
Most common vasculitis in children ?
Henoch-Schönlein purpura
Henoch-Schönlein purpura mechanism ? Presentation ?
IgA immune complex deposition
Presentation : palpable purpura on buttocks and legs GI pain and bleeding Hematuria ( IgA nephropathy ) Occurs following an upper respiratory tract infection
Causes of renal artery stenosis ?
1- atherosclerosis ( elderly males )
2- fibromuscular dysplasia ( young females )
Fibromuscular dysplasia ?
Defect of the blood vessel wall resulting in irregular thickening of large and medium sized arteries especially the renal artery
Intimal plaque of atherosclerosis consists of ?
Necrotic lipid core ( mostly cholesterol ) with a fibromuscular cap ; often undergoes dystrophic calcification
Mechanism of Hyaline Arteriolosclerosis ?
Caused by proteins leaking into the vessel wall producing vascular thickening , proteins are seen as PINK HYALINE on microscopy
Consequence of long standing hypertension or DM
Results in reduced vessel caliber with end organ ischemia ( glomerular scarring that progress to CRF )
Mechanism of Hyperplastic Arteriolosclerosis ?
Thickening of vessel wall by hyperplasia of smooth muscle ( onion skin appearance )
Consequence of malignant hypertension
Results in reduced vessel caliber and end organ ischemia
May lead to fibrinoid necrosis of vessel wall with hemorrhage ( acute renal failure with Flea Bitten appearance )
