churg-strauss syndrome Flashcards
who gets this disease?
middle-aged with a history of new onset or newly worsening asthma. distribution is equal
classic signs and symptoms
asthma is a cardinal feature. early signs are nasal polyps and allergic rhinitis. next is eosinophilia. then vasculitis. this involves tingling, numbness, shooting pains, severe muscle wasting.
what are the bare facts?
someone with asthma, peripheral neuropathy, eosinophilia, transient pulmonary infiltrates, paranasal sinus abnormality, biopsy of a blood vessel that contains extravascular eosinophils.
40-60 have p-ANCA.
worst cases
hypergammaglobinemia, eosinophilic infiltration of the lungs with giant cell reaction leading to granulomatous, necrotic center, inflammation.
what if untreated
death
treatment
prednisone often in combination with methotrexate or cyclophosphamide.
which vessels are affected?
small and medium vessels