Vasculopathies - Spring 2011

8/7/2019 Vasculopathies - Spring 2011

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VasculopathiesDavid Payne PA-C


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Case Study

CC: Jasmine is a 72yo female who presents withheadache and low grade fever


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Case Study

CC: Jasmine is a 72yo female who presents withheadache and low grade feverH PI: Jasmine states that over the last 4-7 days shehas noticed a gradually progressive left-sidedheadache. She describes the pain as piercing, 7/10,and nonradiating. This is unlike any headache she hasexperienced before. She has tried Tylenol for the

headache but this doesnt seem to help. She alsocomplains of mild fever, as well as neck and bilateralshoulder pain and stiffness. No nausea orphotophobia.


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Case Study

What are you worried about when you hear thecombination of headache and neck stiffness? Whatabout when you add fever to the mix?What is it about the HPI that suggests that thispatient is not having a subarachnoid hemorrhage ormeningitis? What should have been included in thenote?Why is this headache not likely a migraine?


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Case Study

PM HWell-controlled HTN, Osteoarthritis (Knees), OsteopeniaPSH: Cholecystectomy (1972)

Meds: Lisinopril/HCTZ (10/25) po daily, Tylenol 500mg ii poq 4-6h prn, C itracal 250/125, ii po bid, 4g Metamucil dailyAll ergies: NKDA

F amily Hx : Sister HTN, Osteoporosis

Soc ial Hx : No ETOH, nonsmoker, widow, eatsbalanced diet, walks daily


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Case Study

Is it refreshing to know that Jasmine has well-controlled HTN? Why?Are we worried about how much Tylenol she is

taking?What is C itracal and why is she taking it? Why notcalcium gluconate?


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Case StudyROS :

G enera l : Has felt fatigue ver last several eeks, n eight changesEENT : No iplopia o r visual isturbances; n o RN o r congestion, no S

Lu ngs: No cough o r yspneaH eart : No C o r palpitati onsGU : No ysuria o r increase frequencyM u sc u loskel eta l : M o rning stiffness an pain ith ove ent o f shoul er

gir le bilaterally an neck f o r several eeks n owN eu r o : No lighthea e ness, weakness, o r tr oubles with speech o r gait

What o you want t o f ocus onf or physical exam?


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STAT labs!

You order some stat labs and receive thefollowing:

CB C normalESR elevated at 63

Whats your diagnosis?

What would you do if yourESR was 38?


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Different manifestati o ns o f the S AMEimmun o lo gic disease pr oc ess!!

Po lymyalgiaRheumati c a (PMR)

Giant-Cell Arteritis(Temp o ral Arteritis)


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Whats going on?

Disease process targeting the synovium,periarticular structures, and medium-to large-

sized arteries ino

lder patients!ino

lder patients!Po lymyalgia Rheumati c a (PMR)Po lymyalgia Rheumati c a (PMR) is theis themilder f o rmmilder f o rm with minimal vasculitis injuryGiant Cell Arteritis (Temp o ral Arteritis)Giant Cell Arteritis (Temp o ral Arteritis) isthe m o re severe f o rmm o re severe f o rm where regionalvasculitis injury dominates the clinical picture


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Epidemiology

PMRPMRAffects mostly white patients2:1 female predominance***Onset after 50 yearsafter 50 yearsIncidence peaks between 70 peaks between 70- -80 years80 years***R isk for developing Giant- Cell Arteritis increased700 in 100,000

GiantGiant- -Cell ArteritisCell Arteritis

Essentially the same epidemiology as PM R with thefollowing exceptionsCondition is 1/5 as prevalent as PM R40-50% of patients give a prior history of PM R (Doesthis mean that only 40-50% ever had PM R prior?)


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Clini c al Presentati o nSubacute or chronic onset of aching and morning stiffnessin the shoulders, hip girdles, neck, and torso in patientsover the age of 50

Symptoms are usually symmetricStiffness at least 30 minutesPain Shoulder pain is more common at presentation (70to 95 percent of patients) than is hip and neck involvement(50 to 70 percent). Pain is worse with movement and may

interfere with sleep.Synovitis and bursitis About half. Palpable synovitisappears to occur in more peripheral joints, such as theknees, wrists, and metacarpophalangeal joints --- usuallymild and nonerosive

PolymyalgiaR heumatica


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Clini c al Presentati o n co ntDecreased range of motionMuscle tenderness Great irony despite name,NOT a prominent feature!Subjective weaknessSystemic signs and symptoms Up to 40 percentof patients experience systemic signs orsymptoms, including malaise, fatigue, depression,anorexia, weight loss, and fever. However, highspiking fever that can sometimes occur in G CA israre in patients who have only PM R



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L ab o rat o ry and ImagingElevated ESR > 40Elevated CR P > 5 (this is more sensitive than ES R )ANA and R heumatoid factor are negativeNormal radiographs of inflamed joints



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Imp o rtant Tidbits***15-30% of patients with PM R have G CA, mustcarefully evaluate for G CADiagnosis

50yo or older at onsetB ilateral aching and morning stiffness (30 minutes ormore) lasting for at least one month (stiffness in 2 orf the 3 following areas: neck or torso, shoulders orproximal arms, hips or proximal thighs)ESR > 40


Some experts advocate prompt response to low dosesteroids as an additional criterion (15-20mg Prednisone

daily significant response usually in 3 days or less)


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Points To ConsiderPoints To Consider

How many people do you think are out there thathave had PMR and never went to a doctor andobtained a diagnosis?How many people do you think are out there thathave had PMR and did go to a doctor but were neverproperly diagnosed?Do you think that successful suppression of PM R decreases the likelihood of developing Giant- Cell

Arteritis?Why am I asking these questions?

How many times do you think PM Ris mistaken as a different diagnosis?

Think about it, what are the symptoms?


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Whats Wrong with this Picture?Whats Wrong with this Picture?


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Clini c al Presentati o nGradual or abruptHA reported in 70% of symptoms, initial symptom in 35%,pain generally piercing or throbbing, unlike that of anyprevious HA (but, not the worst HA ever)May have temporal artery tenderness (but may not!)Polymyalgia symptoms in more than 50% of cases (thismay be the presenting sympt o m!)C o nstituti o nal sympt o ms of fever, fatigue, malaise,anorexia, and weight loss occ ur in the maj o rity o f patients (How is this different than PM R ?)Atypical presentation may include fever of unknown origin(accounts for 15% of FUO cases in patients > 65 years old)

GiantGiant--Cell Arteritis?Cell Arteritis?


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Diagn o sisStandard formal criteria includes the need for threeof the following:

Age at onset > 50New onset or new type of localized HATemporal artery tenderness OR diminished pulseESR greater than 50 mm/hr

Temporal-artery biopsy specimen showingmononuclear infiltration or granulomatousinfiltration with giant cells histologicconfirmation should be obtained EVE N in thesetting of high pretest probability!!



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Management co ntHigh dose daily coritcosteroid therapy (e.g., 60mg prednisone/d)Alternate-day schedules do NOT control the arteritisIn most circumstances, clinical response starts within 24 hours of

initiation of treatmentPatients presenting with ischemic eye symptoms are treated byophthalmologist with high-dose parenteral steroid therapyTapering can be started after clinical and laboratory manifestationshave normalized (goal is maintenance dose of less than 10mg/d andmay take more than 1 year to achieve)Most patients require treatment for 2-3 years

Considering the likelihood of long-termsystemic steroid therapy, what other kind

of treatment should be undertaken at

the onset of treatment for giant-cell arteritis?



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Although scalp tenderness localized to the involved vessel ischaracteristic the inflamed artery may not always be

palpable or tender R emember, formal criteria include either temporal artery tenderness

O R diminished pulseWhen the ES R is normal, a C-reactive protein is worthobtaining as it may be elevated despite a normal ES RNo single laboratory test can rule in or rule out Giant- Cellarteritis, therefore biopsy is usually required!Large-vessel arteritis accounts for 10-15% of cases and putspatients at risk for aortic aneurysm (this is why we shouldavoid using the term temporal arteritis)



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Look at that thing! Why would you have a diminishedpulse with such a large vessel?



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Some Anatomy?Some Anatomy?


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ManagementIf histologic confirmation should be obtained evenif strongly suspecting temporal arteritis, how dowe approach managing the patient?

Do we wait to initiate steroids?

Will initiation of steroids interfere with results of atemporal artery biopsy?



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Management co ntIf histologic confirmation should be obtained evenif strongly suspecting temporal arteritis, how do

we approach managing the patient?Do we wait to initiate steroids?No, the 1 st priority is to establish control of the diseasequickly to limit risk for irreversible blindness!

Will initiation of steroids interfere with results of atemporal artery biopsy?

No, high dose empiric steroid therapy for 1 to 2 weeks priorto biopsy will NOT compromise diagnosis

Why do we even need histologic confirmationif in some cases the diagnosis is obvious?



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R aynauds Phenomenon

An exaggerated vascular response to coldtemperature or emotional stress.Manifested clinically by sharply demarcated color

changes of the skin of the digits due to abnormalvasoconstriction of digital arteries and cutaneousarteriolesRP is co nsidered primary if these sympt o msocc

ur alo

ne witho

ut evidenc

eo

f anyass oc iated dis o rder.S e co ndary RP refers to the presen c e o f thedis o rder in ass oc iati o n with a related illness,

suc

h as systemic

sc

ler o

sis.


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The typi c al atta ck !A typical episode is characterized by:

Sudden onset of cold fingers (or toes) inassociation with sharply demarcated colorchanges of skin pallor (white attack)skin pallor (white attack) and/orc yan o ti c sk in (blue atta ck )

With rewarming, the ischemic phase (white orblue attack) usually lasts for 15 to 20 minutes.The skin subsequently blushes upon recovery,thereby resulting in the erythema of reperfusion.


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White versus B lue Attack!


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C o ld Temperature o r

Emo

tio

nalS

tress

White Atta ck (Pall o r)OR

Blue Atta ck (Cyan o sis)

Erythema o f Reperfusi o n

Lasts15 20minutes


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Primary R aynaud phenomenon , which iscommonly seen in the general population,occurs in otherwise healthy individuals, and

almost always has a benign course.In patients with Systemic Sclerosis, R aynaudphenomenon (which would be termedsecondary) can lead to

UlcerationsDigital InfarctionsGangrene


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TreatmentPrevention (bundling up well against cold temps)Cal c ium c hannel bl ock ers (meds such as nifedipine oramlodipine)Alpha blockers may be given along with or instead of calciumchannel blockers (examples anyone?)Low dose ASA should be usedErectile dysfunction pills?

Treat any associated complicationsDebridement of ulcersAB X for secondary infections


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Paynes Pearls

PMR and Giant- Cell Arteritis are thought to bedifferent manifestations of the same autoimmunedisease process (when you think one, you shouldthink the other)

The signs and symptoms of PM R can be subtleand/or nonspecific and therefore patients may bedifficult to diagnoseSuccessful suppression of PM R with steroid therapydecreases the likelihood of Giant- Cell Arteritis (Ahh,why its important to make the diagnosis, even if much of the time PM R is a self-limiting process!)The first thing you should always be thinking about inthe elderly patient with a unilateral headache is

Giant-Cell Arteritis


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Paynes Pearls

A patient having a Giant- Cell Arteritis may present withpolymyalgia symptoms and not HA!R emember, infection or hemorrhage produces pain thatis acute in onset, severe, generalized, and constant. This isNOT typically the characteristic way that Giant- CellArteritis presentsThe major reason we are so concerned about promptlydiagnosing and treating Giant- Cell Arteritis is to preventits most dreaded complication... blindness

If you have a suspicion, do a work-up!We do NOT wait for histologic confirmation fromtemporal artery biopsy to initiate steroid therapy forGiant-Cell Arteritis!


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Paynes Pearls

You are much more likely to have constitutional symptoms of fever, fatigue, and weight loss in the patient with Giant- CellArteritis versus PM R

R emember that about 15% of cases of Fever of UnknownOrigin in the elderly are due to Giant- Cell Arteritis!

If you have a patient with systemic symptoms and severalunexplained symptoms, screen for autoimmune disease

Vasculopathies - Spring 2011

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