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Polymyalgia Rheumatica, Giant Cell Arteritis, and Polyarteritis Nodosa
Terms in this set (30)
What is giant cell arteritis aka temporal arteritis?
-Narrowing of the lumen
-It can affect any vessel in the body
What is the patho of giant cell arteritis?
-Inflammatory infiltrate contained macrophages and lymphocytes
What are some risk factors associated with giant cell arteritis?
-Age (above 50)
-Mean age is 72
What are some symptoms of giant cell arteritis?
-Temporal artery tenderness
-Decreased pulse in temporal artery
New onset h/a
Jaw pain and claudication
-Visual loss, diplopia, blurriness
What are some complications to giant cell arteritis?
What if the giant cell arteritis affects another vasculature?
-HTN (renal vasculature)
-Claudication (iliac vasculature)
-Abdominal pain (mesenteric vasculature)
What is the most common presentation of giant cell arteritis?
-Unilateral visual loss
What will you find on PE with giant cell arteritis?
-Temporal artery is hard, thickened, absence of pulse, enlarged, tender
-Fundoscopic exam will show a swollen, pale disc, blurred margins
Ischemic optic neuropathy
How do diagnose giant cell arteritis?
ESR is often above 100
-CBC normochormic anemia
-CMP shows elevated AST and alk phos
Temporal artery biopsy in all patient that we suspect
-->if the biopsy is negative and we still have significant concern then we need to do the other side bc of skip lesions-->if it is still negative then the patient may not have giant cell or they may have giant cell involving only the great vessels-->get MR angiogram of aortic, subclavian, carotid, or other large vessel disease-->empiric trial of glucocorticoid therapy may be helpful. If it doesn't resolve then this is highly suggestive of something not giant cell
How do you treat giant cell arteritis?
Initiate systemic glucocorticoids immediately (don't wait for the biopsy results)
Prednisone 40-60mg po QD
If causing visual loss, may need pulse dose of methylprednisolone IV followed by po
Average 9-12 months of prednisone
Initiate low-dose ASA
-Monitor ESR and CRP-->they should start to normalize and that's a sign we can start to taper
What is polymyalgia rheumatica?
-Pain or stiffness, usually in the neck, shoulders, upper arms, and hips, but may occur all over the body
-A relatively common chronic inflammatory condition of unknown etiology that affects elderly individuals
What are risk factors of polymyalgia rheumatica?
-Age (average age of diagnosis is 70)
-If you have giant cell, you're more likely to have it
What are clinical features of polymyalgia rheumatica?
-Subacute or chronic aching and morning stiffness of the neck, shoulder, hip girdle, and torso, that is
Morning stiffness that lasts longer than 45 minutes, they have trouble dressing
(putting on shirts, pulling up pants)
Pain is worse with movement, may interfere with sleep
-We don't see this in the knees
-Non-erosive synovitis (can illicit pain with palpation)
-Pitting edema of hands, wrists, ankles, dorm foot, and carpal tunnel syndrome
-Decreased ROM of shoulders, neck and hip
Subjective weakness, but normal strength on exam
Hand swelling and edema in PMR
-New swelling=put this diagnosis on the top of your list
What does polymyalgia rheumatica look on PE?
-Evaluate truncal, peripheral, and spinal joints
-No tender points (fibromyalgia will have tender points)
-Do a complete MSK exam
How do you diagnose PMR?
-ESR larger than 40 mm/hr, but often it's greater than 100
-CBC thrombocytosis may be present
-ANA and other antibody tests are negative
-Alk phase may be elevated
-Xrays are normal, no joint destruction
Clinical: truncal/shoulder/hip stiffness and pain, morning stiffness longer than 30 minutes, disruption of sleep, joint pain and swelling, muscle pain and subjective weakness, constitutional symptoms, age is greater than 50 yo, B/L aching and morning stiffness, ESR greater than 40 mm.hr, improvement of symptoms with corticosteroids
When evaluating PMR, if absent symptoms of giant cell are absent, should you do a biopsy to make sure?
-Review symptoms of giant cell if you don't remember
How do you treat polymyalgia rheumatica?
-->low dose, typically for 1-2 years (start with
Prednisone 15 mg/day qd)
-Baseline labs before treatment (repeat at 2 months and then every 3 months)-->ESR, CRP, CBC, CMP, UA
-We begin to taper slowly when the stiffness/pain has resolved for 2-4 weeks
-Taper in increments of 2.5mg every 2-4 weeks, but keep in mind they can relapse with tapering
-75% of patients are able to taper off steroids within 2 years, but 25% require life long, LOW (2.5mg) dose steroids
What is SECRET?
-Helps with the diagnosis of PMR
tiffness and pain
onstitutional symptoms, caucasians
What is PolyArteritis nodosa?
-Necrotizing inflammatory lesions that affect medium-sized and small muscular arteries, preferentially at vessel bifurcation resulting in micro aneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and consequently, organ ischemia or infarction
What are some risk for PolyArteritis nodosa?
What is the patho of PolyArteritis nodosa?
-Thickening of inflamed vessel wall, plus intimal proliferation leads to narrow lumen
-This predisposes to thrombotic events and aneurysms from weakened inflamed wall
What are symptoms of PolyArteritis nodosa?
-Constitutional (fatigue, weight loss, weakness, fever)
-Skin: erythematous nodules, purpura, ulcers, vesicular or bullous eruptions
-Can be a normal find
-Lace-like purple finding
Ulcerations and ischemia
-If this is happening on our skins, it's happening in our organs
What are clinical manifestations of PolyArteries nodosa?
Renal (most common organ involved found on autopsy)-->renal insufficiency,
renal artery aneurysm,
, renal HTN
-GI-->ischemic bowel, N/V, melena, bloody or non-bloody diarrhea
-Muscle-->myalgia, muscle weakness
How do you diagnose PolyArteries nodosa?
Biopsy of affected organ
-->most accessible tissues are skin, sural nerve, skeletal muscle, rectum, and testicle
Arteriogram is alternative to biopsy
-->shows multiple aneurysms and artery constrictions
-Labs to rule out other diagnoses-->Creatinine, muscle LFTs, Hep B and C, UA, elevated ESR and CRP
-CXR and blood cultures bc you can't miss endocarditis and sepsis
-Document vasculitis plus 3 of the following:
--Weight loss greater than 4 kg
--Testicular pain or tenderness
--New-onset diastolic BP greater than 90mmHg
--Elevated BUN or creatinine
--Evidence of Hep B
Renal arteriogram of polyarteritis nodosa
How do you treat polyarteritis nodosa?
then taper after significant improvement (usually 4 weeks and then taper slowly. Treatment is usually 9 months)-->they may feel immediate relief
Add cyclophosphamide (Cytoxan-prevents cell division) if not improving on steroids alone but they can't have Hep B
-ACEI for renal HTN
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