7.1 Vasculitis Flashcards
60 y female presents with headache, fatigue, muscle and joint aches, unilateral vision loss, jaw claudication, fever, temporal tenderness. Explain the disease. What labs, and other studies are needed for diagnosis? What is the treatment?
Likely giant cell arteritis caused by inflammation of the walls of the vessels branching from the carotid arteries. Inflammation can lead to occlusion causing ischemia to eye and scalp leading to pain and blindness. Inflammation leads to generalized symptoms.
Labs: ESR, CBC
Dx: biopsy needed, lesions are segmental, looking for inflammation in media and inflammatory cells in the adventitia including lymphs. US may show occlusion or stenosis of temporal or occipital arteries
Tx: Prednisone as soon as Dx is suspected, use for 1-2 months and then a taper, ASA to prevent thrombosis and stroke, vit D and Ca to counter the long term use of steroids and their effects on bones, high risk of blindness without treatment
40 y asian female presents with vertigo and syncope. She also c/o fever, malaise, fatigue. Physical exam shows decreased pulses in carotid and UE. Likely Dx? Workup? Tx?
Takayasu Arteritis: inflammation of the aortic arch and branches
Labs: ESR, biopsy shows inflammatory cells in vessel wall with giant cells and vascular fibrosis
Image: arteiography may show stenosis, CT or MRI can show vessel wall abnormalities
Treatment: corticosteroids, immunosuppression, bypass grafting of vessels
Haw are temporal arteritis and takayasu arteritis the same and different?
Same: large vessel inflammation, giant cells and other cells in wall on biopsy, generalized symptoms from inflammation, treated with steroids
Different: Temporal has vision effects, scalp and temporal tenderness, is in women over 50
Takayasu decreases pulses, is in asian women under 50, can cause MI and CVA
Which vasculitis is associated with HBsAg?
Polyarteritis nodosa
Which vasculitis can kill the patient if untreated?
Polyarteritis nodosa
Young male patient presents with abdominal pain, HTN, melena, hematuria, anemia, neuropathy, palpable purpura, joint pain, and skin ulcers. Dx? Disease process? Biopsy findings? Epidemiology? Organs affected? Workup? Treatment?
Polyarteritis Nodosa
Necrotizing vasculitis leading to ischemia involving many organs but sparing the lungs. Ischemia causes HTN, hematuria, abdominal pain with melena, neurologic disturbances, and skin lesions.
Commonly affects young more than old and men more than women.
Labs: increased WBC, anemia, ESR, proteinuria, hematuria, negative p-ANCA
Angiography would show aneurisms
Biopsy can be helpful, healing with fibrosis will show string of pearls on histology
Treatment: steroids and immunosuppression, fatal if not treated
What vasculitis is more common in men than women?
Polyarteritis nodosa
What disease affects kids less than 4 years old? What nationality is most common?
Kawasaki disease
Often asian kids
3 y asian kid presents with fever conjunctivitis, red palms and soles with eventual desquamation, and large cervical lymph nodes. Dx? Biggest concerns? Workup? Treatment?
Kawasaki Disease: necrotizing vasculitis involving vessels of all sizes. Can involve coronary arteries leading to aneurysm, MI, death.
Labs: can show autoantibodies to endothelial cells
Radiology: echocardiogram shows coronary aneurysms and is most useful when used with dobutamine stress test; angiography can show vessel irregularities
Treatment: ASA to prevent thrombosis, IV gamma globulin, frequently self-limited
What disease causes autoamputation of the digits and is associated with Raynaud’s? What is the treatment?
Buerger Disease: necrotizing vasculitis of the digits associated with heavy smoking. Treatment is smoking cessation.
Describe temporal cell arteritis
Granulomatous vasculitis involving branches of the carotid artery
Common in females over 50. Most common arteritis
Presents with headache, visual changes, jaw claudication, flu-like sx of joint and muscle pain (polymyalgia rheumatica), increased ESR
Biopsy shows giant cells and inflammation
Treatment with steroids to prevent blindness
Describe takayasu arteritis
Granulomatous vasculitis involving aortic arch and branches
Asian females less than 50
Presents with visual and neurological symptoms and weak or absent pulses in UE and decreased carotid pulse, ESR elevated
Treat with steroids
Describe polyarteritis nodosa
Necrotizing vasculitis leading to ischemia that affects most organs except the lungs
Most common in young adults and males over females
Presents with HTN, melena, abdominal pain, hematuria. neuropathy, skin lesions–palpable purpura and ulcers of varying ages.
String of pearls on biopsy
Treat with steroids and cyclophosphamide (chemo agent that decreases immune system), fatal if not treated
Describe Kawasaki disease
Asian kids less than 4 years old
Fever, conjunctivitis, maculopapular rash on palms and soles that eventually desquamates, cervical lymph nodes
Can involve coronary arteries leading to aneurysms, MI, and sudden death
Treat with ASA and IVIG, disease is usually self-limited
Describe Buerger disease
Necrotizing vasculitis involving the digits
Caused by heavy smoking and therefore treated with smoking cessation
Causes ulceration, gangrene and autoamputation of the fingers and toes
