HOW DO WE PREVENT BLINDNESS AND STROKE IN

GCA/LVV?

Scott Pollock, MD

Clinical Professor of Medicine

UW Division of Rheumatology

August 16, 2016

Outline

• PMR and GCA diagnosis, controversies, epidemiology

• GCA in relation to PMR

• Temporal artery biopsy

• Ultrasound in PMR/GCA/LVV

• PMR over- and underdiagnosed—depends on your perspective• Several sets of diagnostic criteria• Is response to steroids specific?• Can patients respond to CS and not have PMR?• Can patients not respond to low dose CS and still have PMR?• Must exclude multiple conditions:

RA, seroneg RA, OA, rotator cuff disorders,malignancy, infection, intracranial tumors

ACR/EULAR: Ann Rheum Dis 2012;71:484–492.

Epidemiology: PMR and GCA

• >50 yo; peak 70-80 yo• PMR is 2nd most common systemic rheumatic disease• GCA is the most common systemic vasculitis• Women: men; 2.5:1• Lifetime risk for GCA in women 1%, men 0.5%• PMR incidence highest in Scandinavian/Northern European

113/100,000 Norway60/100,000 Minnesota13/100,000 Italy

• PMR is 2-3X more common than GCA• PMR present in 40-50% of GCA patients• 20-30% of PMR patients actually have GCA—seen on US and PET-CT• PMR relapses more frequently in those 20-30% with GCA***• ESR <40mm in 5-20%; CRP normal in 1%

Incidence of GCA in Various Countries

Seattle and Washington State: GCA Prevalence

• No figures available

• Minnesota 19/100,000

• England 22/100,000

• Estimate 15-18/100,000 in Washington with population of greater Seattle area 3,800,000

• 600—700 cases of GCA per year

• Vision Loss at 30% results in 180-210 cases of vision impairment per year in greater Seattle area due to GCA

GCA

DIAGNOSIS — The diagnosis of GCA should be considered in a patient over the age of 50 who complains of or is found to have:

• New headaches

• Abrupt onset of visual disturbances—transient precedes permanent

• Symptoms of polymyalgia rheumatica

• Jaw, tongue or extremity claudication

• Unexplained fever or anemia

• High erythrocyte sedimentation rate and/or high serum CRP

Amaurosis fugax

Arterial Ultrasound: Halo effect

GCA: Diagnosis—Classic Approach• Biopsy within 1 week after starting steroids, can bx later• Biopsy—unilateral or bilateral:

5-15% miss with 1 side onlyEULAR bx 1 side; if neg frozen, bx 2nd sideMayo: bilateral bxNo longer perform bilateral bx in most institutions• High likelihood of negative biopsy (95% probability) if:

Normal/mild elevation ESR (<40)Absence of jaw claudicationAbsence of temporal artery tendernessPresence of synovitis, suggests alternative dx• Biopsy negative GCA, approx 5-8% of cases

TA negative but great vessels may be involved (US, MRA, PET)*Empiric trial steroids; resolution sxs within 1 wk*

Difficulties with TA Biopsy

• False negatives: 10-20%, negative biopsy does not R/O GCA

• Sensitivity 30-40%

• 26% of patients with negative bx still were treated with prolonged CS, so was TA bx irrelevant??!!

• Rare side effects

Am Surg. 2012 Dec;78(12):1362-8, Ann Vasc Surg. 2012 Jul;26(5):649-54

Clin Exp Rheumatol. 2015 Mar-Apr;33(2 Suppl 89):S-84-9.

Vision Loss: Anterior Ischemic Optic Neuropathy—AION

• AION most common cause of blindness in GCA

• Prevention of vision loss is essential as there is almost never return of sight –only 4% have some improvement

• One eye involvement predisposes to OU vision loss (13% over 5 yr with Rx, 25-50% without timely Rx)

• Fixed blindness frequently preceded by monocular amaurosis fugax, blurring, or diplopia

• Vision loss rare after 8 weeks CS >40mg qd

• Vision loss in 1 or 2 eyes occurs in 15-35% of most series and usually at presentation before initiation of CS

• Increased risk of bilateral blindness with age >82

Blindness: Can It Be Prevented?

• Must make diagnosis and begin treatment sooner

• Similar to MI and CVA educational programs: public and MDs—GPs, FPs, IM, Ophthalmologists, Neurologists and Vascular Surgeons—must recognize typical and atypical presentations (without HA, fever, PMR, jaw claudication)

• Warning signs and symptoms must be recognized early

• Once vision is impaired, it’s all over!

• Ongoing European attempts to make diagnosis and begin Rx earlier—Fast Track Clinics in England, Norway and Germany

• We should be doing the same!

Estimates of # with GCA & Visual Impairment 2050

Aging population guarantees a gradual increase in prevalence of GCA and Vision loss

Fast Track Clinic & Education: Goals

• Educate public to recognize warning signs and symptoms

• Reduce time from onset of symptoms to seeing any MD

• Reduce time from this MD to one-stop shopping with FTC

• Reduce time from onset of symptoms to initiation of CS

• Patients seen within 24 hours of referral, with initial telephone advice for starting CS immediately if suspicious symptoms

• TA biopsy not always needed (see below)

Technical Difficulties

• Temporal arteries IMT 0.3-0.4mm

• Easy to over or under fill vessel this small with color either creating or obscuring the halo

• Higher frequency probe (22-50mHz) does not require color to evaluate IMT; this type of probe being tested

• Carotid, axillary, subclavian do not require color for diagnosis

GCA: Treatment

•Begin 60-80mg/d prednisone & ASA 81mg qdupon suspicion of GCA

• If visual symptoms (amaurosis or vision loss), consider IV SoluMedrol 1g per day x 3 days

• Taper prednisone after 1-3 months >=40mg/d

•10% reduction every 2 wks

•Remain 10mg qd x 2 mos and then taper 1mg per month for at least 1 year total

• ESR/CRP q 1-3 mos; Rx clinical sxs not lab

Summary

• PMR is very common

• GCA/LVV is the most common vasculitis

• Patients with PMR may really have GCA/LVV

• If PMR is atypical or relapses, re-evaluate for GCA/LVV and other dxs

• Only way to prevent blindness in GCA is early suspicion, dx and Rx

• Fast Track Clinics and a public/physician education program will hopefully lessen risk of vision loss