Vasculopathies Flashcards
What are the constitutional symptoms for vasculopathies?
fever, arthralgias/myalgias, weight loss, malaise
necrotizing arteritis of medium-sized vessels with a predilection for involving peripheral nerves, mesenteric vessels, heart and brain
polyarteritis nodosa
What is polyarteritis nodosa commonly associated with?
vasculitic neuropathy
Infectious disease responsible for 10% of polyarteritis nodosa
Hep B
Typical skin involvement includes: livedo reticularis, subcutaneous nodules, and skin ulcers
polyarteritis nodosa
What is often the most frequent and earliest symptom of polyarteritis nodosa?
Peripheral neuropathy
Would you expect the ANCA results for polyarteritis nodosa to be positive or negative?
ANCA negative
What is required for diagnosis of polyarteritits nodosa?
tissue biopsy or angiogram
What is the mainstay of treatment for polyarteritis nodosa?
high dose steroids initially. Cyclophosphamide for severe disease
Most severe medium sized vessel disease of childhood. Asian children are at higher risk.
Kawasaki’s
Classic h/o includes: high fever, conjunctival injection, mucositis, erythematous rash, and enlarged cervial lymph nodes
Kawasaki’s
What is a major complication of Kawasaki’s?
arteritis of the coronary vessels
Should be performed as soon as Kawasaki’s is suspected, at 2 weeks, and at 6-8 weeks after onset of disease
echocardiogram
Recommended to evaluate arrhythmias, prolonged PR interval, and nonspecific ST and T wave changes of Kawasak’s
EKG and echocardiogram
Patient population with Kawasaki’s more likely to develop coronary artery aneuryms
infants
Affects small arteries. Particularly in the upper and lower respiratory tract, and kidneys. Commonly occurs in the fourth and fifth decade
Wegener’s Granulomatosis
Percentage of patients with Wegener’s Granulamatosis that present with upper or lower respiratory tract symptoms
90%
serum antibody test that is usually elevated with Wegener’s Granulomatosis
C-ANCA
Most common vasculitis in children. Affects small vessels.
Henoch-Schonlein Purpura
Typical features of Henoch-Schonlein Purpura
palpable purpura, abdominal pain, arthritis, hematuria/proteinuria
How soon do most cases of Henoch-Shonlein Purpura resolve?
within one month
What is the main cause of morbidity associated with Henoch-Schonlein Purpura?
renal disease
How frequently should you follow-up a patient with Henoch-Schonlein purpura with UA and BP monitoring?
Weekly for first two months, then monthly or every other month for one year
