IUHS Student-2-Student USMLE Step 1 Recall
Cardiovascular
Vasculitis
Giant
Cell (Temporal) Arteritis
most common of the vasculitis, is an acute and chronic, often granulomatous, inflammation of medium-sized and large arteries...HLA-DR4...
focal granulomatous inflammation...
it affects principally the arteries in the head, especially the temporal arteries, but also the vertebral and ophthalmic arteries...the latter may lead to blindness...often associated with polymyalgia rheumatica...also, it begins in the tunica media...
Pathology - microscopic examination reveals partial destruction of the artery wall by an infiltrate of inflammatory cells, including multinucleate giant cells of both langhans' type (peripheral necklace) and foreign-body type (random distribution of nuclei)...phagocytic cells may contain fragments of the disrupted internal elastic lamina...the lesions often involve long segments of the artery, with interspersed normal segments, which lead to false negative temporal artery biopsies...
Clinical Features - common in older individuals older than 50...fever, fatigue, weight loss, facial pain (painful eating), or headache, vessel itself may be painful to palpatation, diplopia, vision loss...
the temporal artery is a muscular artery and is made up of a thick and complete internal elastic lamina, absent fenestrated elastic membranes in media, thick external elastic lamina, and absent vasa vasorum...
increased Erythrocyte Sedimentation Rate (ESR) to 110 mm/hr...
Takayasu's Arteritis
its a granulomatous vasculitis of medium and larger arteries and as a clinical syndrome characterized principally by ocular disturbances and marked weakening of the pulses in the upper extremities (pulseless disease), related to fibrous thickening of the aortic arch with narowing or virtual obliteration of the origins or more distal portions of the great vessels arising from the arch...
japanese immigrants having trouble reading...
Clinical Features - malaise, low grade fever, weight loss, nausea, cardiopulmonary effects, such as palpitations and shortness of breath...weakening upper extremity with increased pressure in lower extremties...
Pathology - mononuclear infiltrate surrounding the vasa vasorum in the tunica adventitia...this infiltration is followed by diffuse polymorphonuclear leukocyte infiltration and subsequent mononuclear cell influx into the tunica media...
granulomatous vasculitis with massive intimal fibrosis...irregular fibrous thickening of the wall of the aortic arch...narrowing of the orifices of the major arterial branches...
stroke is very possible...
Polyarteritis nodosa (classic)
polyarteritis nodosa is a systemic vasculitis manifested by transmural necrotizing inflammation of small or medium-sized muscular arteries, typically involving renal and visceral vessels and sparing the pulmonary circulation...
glomerulonephritis and vasculitis of arterioles, capillaries, or venules is present...
distribution of lesions, kidneys, heart, liver, and GI tract, followed by pancreas, testes, skeletal muscle, nervous system, and skin...
segmental erosion with weakening of the arterial wall owing to the inflammatory process may cause aneurysmal dilation or localized rupture that is perceived clinically as a palpable nodule and can be demonstrated by arteriography...
Histologically, heavy infiltrate of neutrophils, eosinophils, and mononuclear cells, frequently accompanied by fibrinoid necrosis of the inner half of the vessel wall...inflammatory infiltrate is eventually replaced by fibrous thickening...
Clinical Picture - disease of young adults, malaise, fever, weight loss, hypertension, abdominal pain and melena (bloody stool), muscular aches and pains, peripheral neuritis...
>50% positive for HBsAB...
75% positive for P-ANCA...autoantibody against myeloperoxidase...
Kawasaki Syndrome (mucocutaneous lymph node syndrome)
arteritis involving large, medium-sized, and small arteries (often coronary arteries) that is associated with the mucocutaneous lymph node syndrome, usually in young children and infants (80% or younger)...(japanese americans)...
MI in children is most often related to Kawasaki syndrome...leading to infarction...
Clinical Picture - fever, conjunctival and oral erythema and erosion, edema of the hands and feet, erythema of the palms and soles, a skin rash often with desquamation, and
enlargement of cervical lymph nodes...
Epidemiology - Kawasaki syndrome is the leading cause of acquired heart disease in children in the US...
Pathogenesis - based on immunoregulatory defect characterized by T-cell and macrophage activation, secretion of cytokines, polyclonal B-cell hyperactivity, and the formation of autoantibodies to endothelial and smooth muscle cells, leading to acute vasculitis...
3000 hospitalization every year due to kawasaki...
Wegener
Granulomatosis
chest radiograph reveals a reticulonodular pattern of infiltrates...
Wegener granulomatosis is a necrotizing vasculitis characterized by a triad of:
(1) acute necrotizing granulomas of the upper respiratory tract (ear, nose, sinuses, throat), the lower respiratory tract (lung), or both...saddle nose deformity...
(2) focal necrotizing or granulomatous vasculitis affecting small to medium-sized vessels (capillaries, venules, arterioles, and arteries), most prominent in the lungs and upper airways but affecting other sites as well...
(3) renal disease in the form of focal or necrotizing, often crescentic, glomerulitis...
Pathogenesis - hypersensitivity reaction...immune complexes have been seen in the glomeruli and vessel walls in occasional patients...c-ANCA are present in the serum in 90% of patients with active generalized disease...c-ANCA is an autoantibody against proteinase 3...
patients presenting with hemoptysis usuall have large, serpiginous necrosis with peripheral palisading macrophages...
Morphology - areas are surrouned by a zone of fibrolastic proliferation with giant cells and leukocytic infiltrate and may become cavitary creating a more superficial resemblance to a tubercle...
Clinical Picture - peak incidence is in the age group of the forties...persistent pneumonitis with bilateral nodular and cavitary infiltrates (95%), chronic sinusitis (90%), mucosal ulceration of the nasopharynx (75%), and evidence of renal disease (80%)...skin rashes, muscle pains, articular involvement, mononeuritis or polyneuritis, and fever...
Treatment - immunosuppressive drugs, cyclophosphamide, prednisone, antibacterial drugs...
Buerger Disease (Thromboangiitis obliterans)
Buerger disease is a distinctive disease characterized by segmental, thrombosing, acute and chronic inflammation of medium-sized and small arteries, principally the tibial and radial arteries and sometimes secondarily extending to veins and nerves of the extremities...
thrombosis and microabscesses...
Epidemiology - affects men who are heavy cigarette smokers...
Pathogenesis - often leads to vascular insufficiency...most patients show hypersensitivity to intradermally injected tobacco extracts...direct endothelial cell toxicity induced by or hypersensitivity to some tobacco products...there is an increased prevalence of the human leukoctye antigens -A9 and HLA-B5 in these patients...
Morphology - sharply segmental acute and chronic vasculitits of medium-sized and small arteries with secondary spread to contiguous veins and nerves...
Clinical Features - superficial nodular phlebitis, cold sensitivity of the Raynaud type in the hands, and pain in the instep of the foot induced by exercise (instep claudication)...severe pain, even at rest...chronic ulceration of the toes, feet, or fingers may appear, perhaps followed in time by frank gangrene...
Raynaud Disease
no underlying disease or pathology...
refers to the proximal pallor or cyanosis of the digits of the hands or feet and infrequently the tips of the nose or ears (acral parts)...
Pathogenesis - caused by intense vasospasm of local small arteries or arterioles...Raynaud disease reflects an exaggeration of normal central and local vasomotor responses to cold or emotion...
Clinical Picture - fingers change color in the sequence white, blue, red...
Raynaud phenomenon
refers to arterial insufficiency of the extremities secondary to the arterial narrowing induced by various conditions, including SLE, progressive systemic sclerosis (scleroderma), atherosclerosis, or Buerger disease....
Raynaud phenomenon may be the first manifestation of any of these conditions...
Churg-Strauss
Syndrome
allergic vasculitis...
its a form of necrotizing vasculitis with granulomas of the respiratory tract and asthma...
the disorder is associated with increased serum IgE and peripheral eosinophilia...