Rheum D/O 1 Flashcards
giant cell arteritis affects which vessels (general)
large and medium vessels
MC extra cranial branches of arteries originating at aortic arch
GCA large artery affected (3) (originate at aortic arch)
- Brachiocephalic
- L Common Carotid
- L Subclavian A
pathophys of GCA
leukocyte invasion and immune mediated reaction (CD T4) to elastin causing patchy, concentric intimal proliferation
Thrombosis and vessel occlusion due to immune damage that leads to fibrous, scarring, and stenosis (decreased vasodilation and vasoconstriction)
GCA most commonly affects which SPECIFIC arteries ? why?
- superficial temoral
- occipital
- ophthalmic
- posterior ciliary arteries
highest elastin content
etiology of GCA
unknown
believed to have viral trigger
epidemiology of GCA
individuals age 50+ , mean age 75 (rare before 50)
genetic predisposition (Scandinavian ancestry)
MC in women
DM reduced risk, smoking, arteriosclerosis increased risk
presentation of GCA (5)
- systemic symptoms
- new HA (temporal)
- jaw claudication
- visual changes
- PMR
GRADUAL onset
systemic symptoms of GCA
fever
fatigue
weight loss
pathognomonic for GCA
jaw claudication
severe pain or fatigue after chewing or prolonged speaking
caused by masseter ischemia
GCA on PE
chronically ill
painful, nodular thickening of temporal arteries, superficial erythema and decreased pulses
carotid tenderness, carotid bruits
abnormal visual exam
PMR symptoms (limited ROM)
most feared complication of vision loss
permanent vision loss
MC occurs before starting tx therefore start STEROIDS RIGHT AWAY
lab findings of GCA
reactive thrombocytosis elevated ESR (>100) and CRP
diagnostic procedure of choice GCA
temporal artery biopsy (100% specific but low sensitivity due to patchy)
imaging in GCA
aortic arch and cerebral angiography
evaluates for aneurysm formation (typically aneurysms are asymptomatic)
must also r/o stroke with MRI
tx of GCA
prednisone (40-60 mg daily or high dose)
low dose ASA (ischemic, thrombotic events)
PPI (GI ulceration)
bisphosphonates, vitamin D, Ca (bone loss from steroid)
prednisone dosing GCA
40-60 mg/day for suspected/confirmed GCA - vision loss
high dose parental steroid for visual changes
continued 2-4 weeks and tapered gradually over 9-12 MONTHS
monitoring in GCA management
ESR and CRP monikered over recover while tapering
recurrence of symptoms or new symptoms = flare
evaluation for new aneurysm or dissection (UE ischemia, claudication)
steroid refractory GCA tx
MTX or Tocilizumab (Actemra) IL-6
PMR epi
affects older individuals (50+), median 72, rare <40
caucasians, northern latitudes, F:M 2:!1
PMR affects what?
disease of the joints (synovitis, bursitis, tenosynovitis around joints)
which joints particularly affected by PMR
shoulder and hip MC
also knees, metacarpal phalangeal joints, wrists
pathophys PMR
systemic inflammation and activation of immune cytokines
possibly due to a viral activation
pt presentation of PMR
acute or chronic onset of proximal myalgia of hip and shoulder girdles
MORNING STIFFNESS
typically progress to bilateral
describe great difficulty rising from chair or lifting arm above shoulder due to pain
PMR associated illnesses
GCA
pts are at risk for GCA development and should seek medical attention if they develop symptoms
PE of PMR
systemic symptoms (50%+) - LOW fever, weight loss, depression, malaise
appear ill and fatigued
NO muscle atrophy but pain with movement
PMR work up
- ESR/CRP (ESR > 40)
- CBC (thrombocytopenia)
- CK normal
- Autoantibody testing ALL NORMAL
normal radiographs
how is PMR diagnosed
clinically
right population (>50) + absence of abnormal fingings + rapid relief with steroid initiation
tx of PMR
corticosteroids in low dose (15mg/day)
symptoms should improve dramatically after 48-72 hrs (if not, consider alternative diagnosis)
biologics are ineffective
steroid taper in PMR
SLOW taper
typically steroid dependent for 3+ years
PMR prognosis
relapse occurs in 25% of pts, MC due to rapid tapering
