CM III - Polymyalgia Rheumatica & Giant Cell Arteritis/Temporal Arteritis

What are the clinical manifestations of GCA/TA?

- HA - Scalp tenderness - Jaw claudication - PMR sx (SECRET) S—Stiffness and pain (neck, shoulders, and hip girdle) E—Elderly Individuals (>50) C—Constitutional symptoms, caucasians R—"Rheumatism", arthritis E—Elevated ESR (and CRP) T—Temporal arteritis - Vision loss, diplopia, Amaurosis fugax - irreversible blindness

Initiating _____ is both diagnostic and tx for PMR

10-20mg prednisone adjust ever

If a pt cannot tolerate prednisone for PMR, what is 2nd line? what is 3rd line?

2nd = MTX 3rd line = Imuran

What defines the 1990 ACR Criteria of GCA dx? What is required to make a presumptive dx?

3 out of 5 criteria are required to make DX (bx is still required to confirm)

What is the criteria for dx'ing PMR?

> 50 years bilateral/proximal sx in 2 of 3 areas (neck, shoulders, proximal regions of the arms, hip girdle, or proximal aspects of the thighs) x 2 weeks elevated ESR/CRP Sx better within 3 days with prednisone at a dose of 10-20 mg/d

What is the murmur seen in TA/GCA? Where are bruits heard?

AR Subclavian/clavicle - bruits

A 56-year-old woman presents with c/o sudden onset of pain and stiffness in her shoulders bilaterally and hips over the past 2 days to the point that it is difficult to get out of bed and get dressed. She denies headaches or visual changes or loss. On PE, she has good muscle strength but difficulty raising up from the chair and abducting her arms bilaterally. Visual acuity and visual fields are intact. She has no temporal artery tenderness or scalp tenderness. Which lab test would be helpful to confirm your suspected diagnosis? •A. Antinuclear antibody •B. ESR •C. Rheumatoid factor •D. Anti-mi2 antibody •E. Anti-SSA antibody

B

What is the gold standard and is necessary for an official dx of GCA/TA?

BX •Granulomatous inflammation or Mononuclear cell inflammation •Multinucleated giant cells •Typical histopathologic finding: non-necrotizing panarteritis

What adjuvant tx should be given w/ prednisone for PMR?

Bisphosphonates, Ca+ and vitamin D •BMD scan

You are treating a patient with GCA with steroids and shortly after you lower the dose of the steroid she complains of some increasing jaw symptoms and scalp tenderness? Which of the following would you do next? A.Add a DMARD B.Continue to decrease the dose and check labs C.Increase the steroids back to the previous dose and check labs D.Leave dose as is and check labs

C

A 66-year-old woman presents to her PCP for evaluation of new onset headaches around her temples with tenderness of her scalp when she washes or brushes her hair, fatigue, low grade fevers, anorexia, and weight loss. Her CRP and ESR are markedly elevated. Which of the following is the Gold standard for diagnosing this suspected condition? A.CT scan of the brain B.Elevated ESR C.MRI of the brain D.Temporal artery biopsy

D

Which of the following best describes the age group that will usually develop symptoms of PMR? A.15-30 y/o B.25-40 y/o C.30-45 y/0 D.50-70 y/o

D

A patient presents with abrupt onset of bilateral shoulder pain and stiffness with difficulty getting dressed and combing her hair. She denies headaches, scalp tenderness or visual changes. Her ESR is > 50 mm/hr and CRP is > 5mg/L. What is the treatment of choice for this patient with your suspected diagnosis? A.Antibiotics B.Biologics C.DMARDs D.NSAIDs E.Steroids

E

Which of the following area is usually affected in patients with PMR? A.Calves B.Diaphragm C.Hands D.Knees E.Shoulders

E

Which of the following sign or symptom that is one of the most feared complications associated with GCA? A.Hearing loss B.Distal muscle weakness C.Loss of gag reflex D.Trouble swallowing E.Vision loss

E

Your patient who you suspected of GCA was referred to the general surgeon for a temporal artery biopsy. In the meantime, what is the treatment of choice for this patient while you await the results of the biopsy? A.Antibiotics B.Biologics C.DMARDs D.NSAIDs E.Steroids

E

Similar to PMR, as you taper the dose of prednisone when tx'ing GCA, what do you need to monitor?

ESR/CRP

Define giant cell arteritis/temporal arteritis

It is a vasculitis affecting medium and large sized vessels in pts over 50.

Should you wait to start a pt on prednisone while awaiting biopsy of temporal artery for suspected GCA?

NO - start immediately

Is imaging necessary for PMR?

No

•About 50% of pts with GCA have ____!

PMR

You are seeing a 50 year old pt who complains of morning achiness and stiffness of the shoulders arms and hip girdle, has an elevated CPR/ESR? What is the likely dx? What is the tx?

PMR --> tx 10-20mg prednisone Dx criteria- > 50 years bilateral/proximal sx in 2 of 3 areas (neck, shoulders, proximal regions of the arms, hip girdle, or proximal aspects of the thighs) x 2 weeks elevated ESR/CRP Sx better within 3 days with prednisone at a dose of 10-20 mg/d

What is the tx for PMR?

Prednisone 10-20 mg/day w/ taper --> 15-20mg x 4 weeks --> dec by 2.5mg q4-6 weeks until at 10mg --> dec by 1-2.5mg to 5mg --> 5mg x 3 months --> taper 1mg q2-4wk while monitoring ESR/CRP

What pneumonic is used to desceribe the sx of PMR. What do they mean?

SECRET •S—Stiffness and pain (neck, shoulders, and hip girdle) •E—Elderly Individuals (>50) •C—Constitutional symptoms, caucasians •R—"Rheumatism", arthritis •E—Elevated ESR (and CRP) •T—Temporal arteritis

Who is GCA/TA commonly seen in?

Scandinavian countries and northern European descent women over 50 years •About 50% of pts with GCA have PMR

What are the clinical manifestations of polymyalgia rheumatica

Symmetrical shoulder girdle pain/difficulty grooming Neck and pelvic girdle pain and stiffness Muscle tenderness of deltoid, biceps, triceps, and quadriceps - NOT WEAKNESS Pitting edema --> hands and feet Carpal tunnel syndrome d/t wrist swelling •Constitutional Sxs: malaise, low-grade fever, fatigue, depression, anorexia, wt loss

What are the PE findings from GCA/TA?

Temporal Artery --> tenderness, pulselessness, enlarged/thick Scalp Tenderness Asymmetric blood pressures Bruits (subclavian & clavicle)/Murmur (AR) Visual field loss - ischemic optic neuropathy. MSK - PMR sx (SECRET), distal synovitis, pitting edema

what systemic complication do pts w/ PMR need to be routinely monitored for? How do you assess for this?

Temporal arteritis & giant cell arteritis •Jaw claudication •Temporal artery tenderness •Scalp tenderness •Ask about HAs •Ask about visual changes --> eye exam - acuity and fields

Besides prednisone, what other tx should rx'd to pts w/ GCA/TA?

bisphosphonates, Ca+, Vit D, ASA

Who is polymyalgia rheumatica most commonly seen in?

caucasian women over 50 "i've never seen a case of PMR under 50"

Describe the findings from an MSK exam in a pt w/ PMR?

difficulty actively abducting the arm beyond 90 degrees decreased shoulder, cervical and hip ROM but muscle strength is normal Soft tissue swelling /synovitis can occur and usually mild in the wrists, and MCP joints and small knee effusions NOT WEAKNESS

What is the only lab abnormality seen in PMR?

elevated ESR/CRP

If while taper prednisone in a pt w. PMR they have a flare or ESR/CRP increase what should you do? TAPER Prednisone 15-20 mg/day w/ taper --> 15-20mg x 4 weeks --> dec by 2.5mg q4-6 weeks until at 10mg --> dec by 1-2.5mg to 5mg --> 5mg x 3 months --> taper 1mg q2-4wk while monitoring ESR/CRP

temporary increase in the prednisone. Every time you adjust prednisone in these pts check ESR/CRP

Polymyalgia rheumatica etiology

unknown

What are the constitutional sx seen in PMR?

•Constitutional Sxs: malaise, low-grade fever, fatigue, depression, anorexia, wt loss

What are the non-specific sx assoc w. GCA/TA?

•Cough •Throat pain •Tongue pain •Aortic branch vessel involvement •Arm or leg claudication •Peripheral synovitis and pitting edema

What are the common arteries affected in GCA/TA?

•Extracranial branches of carotid arteries •Primary branch arteries of aorta

What is seen on bx from TA/GCA?

•Granulomatous inflammation or Mononuclear cell inflammation •Multinucleated giant cells •Typical histopathologic finding: non-necrotizing panarteritis

What is the rx that should be prescribed while awaiting bx for suspected GCA/TA?

•Maintain initial prednisone dose X 4 weeks (60 mg-80 mg)-1mg/kg/day - +/- bisphosphonates, Ca+, Vit D, ASA If unable to tolerate roids or unable to taper = MTX or tocilizumab

What are lab findings seen in GCA/TA?

•Marked elevation of ESR -- > ≥50 mm/hr •↑ CRP •CBC with diff: Thrombocytosis, normochromic anemia •Elevated ALP/ALT/AST

Diagnosis of GCA should be considered in a patient ≥ 50 yrs who complains of?

•New HA •Abrupt onset of visual disturbances, espec transient monocular visual loss •Jaw claudication •Unexplained fever, other constitutional symptoms and signs, anemia •Elevated ESR and CRP (especially if pt has PMR)

What is the initial tx for GCA/TA? (not rx's)

•Recommend referral ASAP to: immediately start prednisone Rheumatologist General surgeon for temporal artery biopsy Ophthalmologist for eye exam.

Define polymyalgia rheumatica

•Systemic inflammatory disease with symmetric, proximal achiness and stiffness in the neck, shoulders and hip girdle areas that are usually much worse in the mornings and after prolonged rest. NOT WEAKNESS (like PM/DM) --> achiness and stiffness Associated constitutional sxs are common.