Vasculitis Flashcards
Infectious causes of vasculitis
Fungal (Aspergillus), Bacterial (Pseudomonas), Viral (CMV)
Epidemiology of Temporal (Giant Cell) Arteritis
Elderly (>50) white females of Northern European ancestry
Why must you be elderly to get Temporal (Giant Cell) Arteritis?
Giant cells attack only CALCIFIED internal elastic lamina in arteries
Most severe complication of Temporal (Giant Cell) Arteritis?
Blindness (why you must make the dx)
Symptoms/Signs of Temporal (Giant Cell) Arteritis?
Headache, Swollen tender artery, visual disturbances, jaw claudication
Main immune cells involved in Temporal Arteritis?
Dendritic Cells and Th1 cells
Tx for Temporal Arteritis?
Corticosteroids and anti-TNF therapy, almost complete reduction in Th17 cells
MMPs up-regulated in Temporal Arteritis?
MMP-2 and MMP-9, destroy the elastin
What must you know about doing a biopsy for Temporal Arteritis?
The disease can be segmental so false negatives are possible (commonly only lymphocytic on biopsy)
Blood test important for dx of Temporal Arteritis?
ESR, normally >100
Epidemiology of Takayasu Arteritis?
Young east Asian females and children
Most important clinical manifestations of Takayasu Arteritis?
Pulseless upper extremity, difference in bilateral blood pressures, visual defects
Pathogenesis of Takayasu Arteritis?
Necrotizing granulomatous vasculitis of the Aortic Arch and its vessels–> Aortic tear, dissection, or rupture
Cardiac complication of Takayasu Arteritis?
Cardiac Tamponade
Tx for Takayasu Arteritis?
Corticosteroids and Cytotoxic immunosuppression
Epidemiology of Polyarteritis Nodosa?
White males in their 40s
Important presentation of Polyarteritis Nodosa?
Neuropathy (sensory or motor like wrist/foot drop), Renal involvement with HTN
Effect of Polyarteritis Nodosa on the Lungs?
None
Skin change seen with Polyarteritis Nodosa?
Livedo Reticularis (mottled discoloration of the skin)
Most distinguishing microscopic characteristic of Polyarteritis Nodosa?
Fibrinoid Necrosis, neutrophilic in the acute phase
HBsAg (Hepatitis B) is associated with? (not diagnostic)
Polyarteritis Nodosa
Tx of polyarteritis Nodosa?
Corticosteroids and cytotoxic immunosuppression (if necessary)
Angiogram appearance of Polyarteritis Nodosa?
“Beads on a String”, focal vasculitis producing nodular lesions
Epidemiology of Kawasaki Disease?
East Asian children < 5 (usually presents around age 1)
Symptoms/Signs of Kawasaki Disease?
Polymorphous rash, cervical lymphadenopathy, erythema/edema/desquamination of hands and feet (KNOW THIS), and “strawberry tongue” glossitis (KNOW THIS)
Pathophysiology of Kawasaki Disease?
Necrotizing vasculitis of the coronary arteries, patients get coronary aneurysms, MIs, and SCD if untreated
Tx for Kawasaki Disease?
ASA and IVIG
What is Reye syndrome?
Rare condition causing cerebral edema and hepatic steatosis when taking ASA for viral illness (he didn’t teach us this, but “Reye Syndrome” is in his slides)
Prognosis for Kawasaki Disease?
Good if treated, most aneurysms resolve
Pathophysiology of Microscopic Polyangiitis?
Necrotizing fibrinoid vasculitis of capillaries, small arterioles, and venules (mostly glomeruli and pulmonary capillaries)
How can you differ Microscopic Polyangiitis from Wegner’s granulomatosis?
Microscopic Polyangiitis has no nasopharyngeal involvement
Antibodies seen in Microscopic Polyangiitis?
p-ANCA (anti-Myeloperoxidase)
Epidemiology of Wegner’s Granulomatosis?
Whites in their 40s, men and women equally affected
Pathophysiology of Wegener’s Granulomatosis?
Necrotizing granulomatous vasculitis of arteries and veins in the respiratory tract (+ hemoptysis) and kidneys
Common findings of physical exam for Wegener’s Granulomatosis?
Mucosal ulcers, nasal septal cartilage destruction, and other nasopharyngeal lesions (distinguishes it from micorscopic polyangiitis)
Tx of Wegener’s Granulomatosis?
Corticosteroids and Rituximab (B cell involvement)
Triad seen in Churg Strauus Syndrome?
Asthma, Vasculitis, and Eosinophila (KNOW THIS)
Antibody seen in Churg Strauss Syndrome?
p-ANCA (not dx)
Epidemiology of Buerger Disease?
Young male smokers of Middle Eastern and South Asian ancestry
Pathophysiology of Buerger Disease?
inflammatory thrombosing vaso-occlusive disease of arteries/veins of the limbs
Tx for Buerger disease?
Stop smoking and amputation for gangrene
Pathophysiology of peripheral arterial disease?
Chronic atherosclerotic occlusive disease of large/medium arteries of the legs
Symptoms of peripheral arterial disease?
Intermittent claudication (leg pain with exercise, relieved by rest). Mainly in the calf muscles (femoral/popliteal arteries). Ulcers and pain at rest–> severe disease
The signs/symptoms of peripheral arterial disease (the 5 Pee Pees)
Pain, pallor, paralysis, paresthesia, and pulselessness
Tx for peripheral arterial disease?
Exercise, especially walking. Helps to develop collateral perfusion
Tx for acute arterial occlusion?
Emergency surgery
