Vaculitides Flashcards
What is Kawasaki disease?
Kawasaki disease is an acute febrile vasculitis of children, marked acutely by fever, rashes, lymphadenopathy, and irritability and chronically by late cardiac complications, including coronary artery aneurysms and myocardial infarction. Fever is present on the first day of the illness and may last from 1 to 3 weeks. The child with Kawasaki disease is irritable, lethargic, and has bilateral congestion of the conjunctivae. The oral mucosa may turn deep red, and the lips often become dry and cracked. It is common for the affected child to have unilateral cervical lymphadenopathy. A strawberry tongue is a prominent sign, as is redness and peeling of the skin of the hands and feet. The disease is rarely fatal in the acute phase, but children may die suddenly from coronary artery disease some years later. This disease was previously called mucocutaneous lymph node syndrome. Cervical lymph nodes are most often the ones enlarged.
Epidemiology: In 80% of children, diagnosis takes place before age 5, and usually the younger the age at onset, the more severe the disease. The disease is rare after age 8. It occurs more commonly in Asian children than in other ethnic groups. In Japan, there are about 5000 to 6000 cases annually. By comparison, about 4000 cases occur annually in the U.S.
Diagnosis: Because of the similarities of this disease to others, e.g., scarlet fever and toxic shock syndrome, diagnostic criteria are strict. There must be fever and at least four of the following five findings: conjunctivitis; oral lesions like those described above; redness, swelling, and peeling of the fingers and toes; rash similar to that described above; cervical lymphadenopathy. The erythrocyte sedimentation rate (ESR) is elevated.
Etiology: The cause is unknown, but both infectious and immune mechanisms have been proposed.
Complications: Formation of giant aneurysms of the coronary artery (esp. in infants and very young children) is the major complication and can lead to sudden death or myocardial infarction later in life. Mortality from the disease is about 1%. Other findings may include arthritis, otitis media, diarrhea, uveitis, pyuria (sterile), and hepatic dysfunction.
Treatment: If given within 10 days of onset of fever, high-dose intravenous immunoglobulin (IVIG) therapy over 12 to 24 hr can dramatically relieve the symptoms and prevent coronary artery dilation. Daily aspirin therapy has traditionally also been given to decrease the risk of coronary artery dilation, but its safety (given the risk of Reye’s syndrome) and its effectiveness have been questioned. Neither antibiotics nor high-dose corticosteroids are effective. Frequent follow-up care, including repeat evaluations to detect or monitor heart disease, is essential.
What is arteritis?
Arteritis is inflammation of an artery.
What is Takayatsu arteritis?
Takayatsu arteritis is a rare vasculitis of the aorta and its branches, marked by in- flammatory changes in the large arteries. Blood flow through those arteries is limited, esp. to the arms or head of affected persons. The disease, which is found most often in young women of Japanese descent, produces symptoms such as dizziness or arm claudication. Those affected usually have markedly reduced blood pressures or pulses in one or both arms.
What is giant cell arteritis (temporal arteritis)?
Giant cell arteritis (temporal arteritis) is a systemic inflammatory vasculitis of medium and large sized arteries that leads to ischemic optic neuropathy and potentially irreversible vision loss. Giant cell arteritis is commonly associated with polymyalgia rheumatica.
The histopathological finding of giant cell arteritis is transmural granulomatous inflammation with fragmentation of elastic fibers.
What is polyartertis nodosa?
Poly arthritis nodosa is widespread inflammation of adventitia of small and medium-zines arteries with impaired function of involved organs.
What is Chung-Strauss syndrome?
Churg-Strauss syndrome is a rare systemic vasculitis affecting the respiratory, musculoskeletal, cardiac, and peripheral nervous systems. It typically develops in patients with a history of asthma or allergy and is marked by hypereosinophilia.
What is granulomatous is with polyangitis (Wegener granulomatosis)?
Granulomatous with polyangitis is a systemic necrotizing vasculitis that is marked by pneumonitis and glomerulonephritis and sometimes affects small and medium-sized blood vessels throughout the body. The average age of onset is 40; the disease affects men more often than women.
Etiology: The precise cause is unknown. Autoantibodies (specifically antineutrophil cytoplasmic antibodies) have been identified in the blood of approximately 90% of patients. Granulomas may be present in the lung, upper respiratory tract, and small arteries and veins. Localized or diffuse inflammatory patches are seen in the glomerular capillaries of the kidney.
Symptoms: Chronic pneumonitis and glomerulonephritis are the most prominent signs; ulcerations of the nasopharyngeal mucosa also are common. Other signs and symptoms include muscle and joint pain, skin rashes, fever, and neuropathy.
Treatment: Suppressive immunotherapeutic drugs such as cyclophosphamide and corticosteroids are used to control the disease. Trimethoprim-sulfamethoxazole may prevent relapses. There is a 1-year, 80% mortality rate in untreated patients; when treatment is effective, patients can live normal lifespans. Those with diffuse glomerular damage may develop chronic renal failure.
What is thromboangiitis obliterans (Buerger disease)?
Thromboangiitis obliterans (Buerger disease) is a chronic, recurring, inflammatory, but nonatheromatous vascular occlusive disease, chiefly of the peripheral small and medium-sized arteries (and sometimes veins) of the extremities, causing decreased blood flow to the feet and legs. The disease occasionally affects the hands and causes painful ulceration of the fingertips.
Incidence: It is diagnosed in approximately 1 in 8000 adults. The disease is seen most commonly in males 20 to 40 who smoke cigarettes or chew tobacco.
CausesL: Tobacco use is a key element in Buerger disease. Some evidence links Buerger disease with periodontal infection.
Symptoms: Symptoms include paresthesias of the foot, easy fatiguability, and cramps in the foot and leg. In patients with severely limited blood flow to the legs, skin ulceration or moist gangrene of the extremities may develop.
Diagnosis: Buerger disease is agnosed in people with ischemic limb symptoms. The patient should not have other known causes for ischemia of the limbs, such as diabetes mellitus, embolic occlusion of blood vessels, or autoimmune disease.
Treatment: Absolute, permanent abstinence from tobacco in all forms is crucial. The patient should avoid excessive use of the affected limb, exposure to extremes of temperature, use of drugs that diminish the blood supply to the extremities, trauma, and fungal infections. Aspirin and vasodilators may be prescribed. If gangrene, pain, or ulceration is present, complete bedrest is advised with a padded foot-board or bed cradle to prevent pressure on the extremities; if these are absent, the patient should walk at a comfortable pace for 30 min twice daily. For arterial spasm, blocking of the sympathetic nervous system by injection of various drugs or by sympathectomy may be done. Amputation is sometimes needed to remove infarcted tissue.
What is thrombophlebitis?
