Polymalgia Rheumatica

Question 1

Definition

Answer 1

Various criteria published, but diagnostic features; age >50y, increase ESR/CRP, bilateral aching or pain, tenderness, and am stiffness (>30-45 min) of proximal muscles groups (shoulder/upper arm»neck and/or pelvic girdle) for > 2weeks, rapid response to steroids, and exclusion of other conditions

Question 2

Other symptoms

Answer 2

Fatigue, low-grade fever, mild synovitis (MCPs/knees/wrists), anorexia, wt loss, distal extremity swelling (usually unilateral)

Question 3

Muscle weakness

Answer 3

Not characteristic of the disease (may be limited by pain)

Question 4

Epidemiology

Answer 4

Most common inflammatory rheumatic disease in elderly white pts; typically occurs in M:F 1:2-3; incidence in pts >50y, approx. 50/100,000; average age of dx >70y

Question 5

Ddx

Answer 5

RA, spondyloarthropathy, pseduogout, dermatomyositis/polymyositis, infection (eg viral syndrome, SBE), SLE, endocrinopathy (eg hypo- or hyperthyroidism), OA, rotator cuff d/o, fibromyalgia, malignancy (MM)

Question 6

Association with giant cell arteritis

Answer 6

GCA is a large- to medium-sized arteritis that involves branches of the proximal aorta; p/w jaw claudication, scalp tenderness, blurred vision, new HA; may occur w/PMR or in isolation; PMR and GCA hypothesized to be different manifestation of one disease process; 16-30% of pts w/PMR have GCA; 40-60% of pts w/GCA have PMR

Question 7

Exam

Answer 7

Decrease active and passive ROM in affected joints; proximal joint swelling; swelling of hands and/or feet; nonerosive peripheral arthritis; assess tenderness in scalp and temporal artery

Question 8

Workup

Answer 8

check ESR and CRP; IL-6; if available; check TFTs, Chem-12, CBC w/diff, UA; RF and anti-CCP typically -; ANA in elevated titer not assoc w/PMR; PMR assoc w/ anemia of chronic disease; CPK should be nl: nl ESR does not exclude PMR (ESR <50 mm/h seen in 10%)

Question 9

Radiographs

Answer 9

Not routinely ordered and rarely helpful; may aid dx if nl ESr; MRI or U/S can demonstrate synovitis; joint erosions are not assoc w/PMR

Question 10

Giant cell arteritis

Answer 10

Screen by H and P; no role for temporal artery bx w/o sx or suspicion for GCA (eg very high ESR or significant systemic manifestations)

Question 11

Steroids

Answer 11

Mainstay of Rx w/initial dose of 15mg/d (maximum 20-30mg/d) of prednisone or equivalent; anticipate initial improvement within 24-48 h and continued improvement over subsequent wks; failure to respond to steroids should raise suspicion for alt dx (eg vasculitis); higher doses of steroids may be used to treat flares; check BMD, A1c, lipids, PPd (if risk factors present) for pts on long-term streroids to screen for glucocorticoid-related osteoporosis, DM@

Question 12

Steroids duration

Answer 12

Steroid Rx often continues for 2-3 y w/slow dose decrease guided by inflammatory markers (ESR, CRP, +/- IL6) and sx; maintenance dose typically 2.5-5mg/day prednisone: 50% patient relapse

Question 13

Steroid sparing agents

Answer 13

Trials, case-series, and reports describe successful use of MTX, leflunomide, tocilizumab, and TNF alpha blockade.