Cyclophosphamid produces a striking involvement w/ both complete remissions and substantial disease-free intervals in most pts
W/o tx pts die w/in a year of onset
What is Churg-Strauss Syndrome and what is its other name?
"Allergic Granulomatosis and Angiitis"
Systemic vasculitis w/ prominent eosinophilia that occurs in young ppl w/ asthma
Widespread necrotizing vascular lesions of the small and medium sized arteries, arterioles, and veins of the lungs, spleen, kidney, heart, liver, and CNS
What is the pathology of Churg-Strauss Syndrome?
Lesions are characterized by granulomas w/ an intense eosinophilic infiltrate in and around blood vessels, resulting in fibrinoid necrosis and thrombosis
What is the ANCA involvement for Churg-Strauss Syndrome?
Both C-ANCA and P-ANCA in 2/3 of pts
What is the tx for Churg-Strauss Syndrome?
Untreated pts have a poor prognosis
What is Thromboangiitis Obliterans and what is another name?
Occlusive, inflammatory disease of medium and small arteries in the distal arms and legs that occurs almost exclusively in middle-aged heavy smokers (stop smoking and there will be a remission; mechanism unknown)
What are the general features of Thromboanglitis Obliterans?
Upper extremity affected lots
What is the etiology of Thromboanglitis Obliterans?
Certain byproducts of tobacco elicit antibodies that can induce inflammation
Greater frequency in Japan, Israel, India (maybe genetic factors)
What are the microscopic pathology of Thromboangiitis Obliterans?
Earliest changes is an acute inflammation of medium-sized and small arteries
PMN infiltrates extends to involve neighboring veins and nerves
What are the complications of Thromboangiitis Obliterans?
Involvement of the endothelium in the inflamed areas leads to thrombosis and obliteration of the lumen
Small microabscesses of the vessel wall, w/ neutrophils and giant cells are prominent
Early lesions can be severe enough to result in gangrene of the extremities, the only tx being amputation
Who is more likely to get Varicose Veins?
Individuals from families known to have a connective tissue weakness, and family histories
Professions that require long hours of standing
Pregnant women - ↑ BP in the pelvic veins compressed by the pregnant uterus and the subsequent stagnation of blood in the leg veins. Thrombotic occlusion of the veins (thrombophlebitis), prevents the outflow of venous blood, and also predisposes pts to varicosities
What are the clinical findings of Thromboangiitis Obliterans?
Symptoms usually start between the 25-40 yo
Claudication (cramping pains in the muscles following exercise, which are quickly relieved by rest)
Pts often present w/ painful ulcerations of the digits, which can progress to the destruction of the tips of the involved digit
Continuation of smoking may lead to loss of both hands and feet
What is the etiology of Varicose Veins?
Back pressure from the failing heart may ↑ the pooling of blood in the veins, w/ stagnation of blood
Dilated veins - veins have valves that oppose back flow of blood, but these venous valves easily become incompetent when the veins dilate
What is the pathophysiology of Varicose Veins?
Once leg veins become dilated, they tend to remain so, and become varicose
Slow flow of blood to the veins predisposes an individual to clotting
Clotting is even more likely in dilated, varicose veins and those in which the back pressure from the failing heart causes stagnation
What are complications of Varicose Veins?
Blood flow in the varicose veins are turbulent and slow, favoring clotting and thrombosis that may become loose and embolize to the lungs
Pooling of blood in the lower parts of the legs causes blood to leak from distended, small capillaries and veins into the tissues
What are clinical features of Varicose Veins?
Brownish discoloration of the skin attributed to the accumulation of blood pigments (hemosiderin) in the subcutaneous tissue
Skin is dry, scaly, shows small pinpoint hemorrhages from ruptured capillaries called "Stasis Dermatitis"