GCA PMR Flashcards
What is Polymyalgia Rheumatica?
Literally “Poly” – many/multiple, “myalgia” – achy muscles
Term was first used in 1957 to describe an inflammatory condition which is characterized by bilateral pain and morning stiffness of the shoulder, neck, and pelvic girdle
What is the epidemiology of Polymyalgia Rheumatica?
Rare under the age of 50; the incidence rises with age, peak between the ages of 70-80
The incidence of disease in patients over 50 is about 12-50 per 100,000
Two to three times as common in women than men
Second most common rheumatic disease in terms of lifetime risk (RA is most common)
More common in Scandanavian countries and those of Northern European descent; much less common in Asian, Latino, and African-American populations
What is the etiology of polymyalgia rheumatica?
As with most rheumatic disease, overall unknown.
Genetics: Modest degree of familial aggregation. 60% of those affected have a HLA-DRB1*04 haplotype variant. Patients with PMR and GCA share the sequence polymorphism.
Environmental triggers.
Infection: Increased prevalence of antibodies to respiratory syncytial virus and adenovirus in PMR; association between increased incidence of the disorder during epidemics of Mycoplasma pneumoniae, Chlamydia pneumoniae and Parvovirus B19.
Describe the classic presentation of PMR
Age > 50
Symmetrical aching and stiffness of the shoulders, hip girdle, neck, torso
Pain usually severe in intensity, in proximal parts of the extremities, often accompanied by limitations in ROM, particularly the shoulders
Fairly abrupt onset
Morning stiffness, severe, lasting for about an hour
Normal strength
Systemic symptoms: fatigue, weight loss, fever, and sweats
What are some atypical presentations of GCA?
ESR less than 40 mm/hour
Peripheral synovitis (think about seronegative RA)
Sternoclavicular synovitis
Carpal tunnel syndrome
Asymmetric pain (though can happen at onset, then become symmetric)
Describe PMR 2012 ACR criteria for classification
Age >50 b/l shoulder pain elevated CRP and/or ESR -morning stiffness >45 mins -hip pain or limited ROM -absence of RF or ACPA -absence of other joint involvement -at least one shoulder with bursitis/synovitis and at least 1 hip with synovitis or bursitis
What are diagnostic testing for PMR?
ESR: Usually high and above 40 mm/hour, although up to 20% of patients may have a normal ESR
C-reactive protein, usually elevated: More sensitive than ESR
IL-6 levels: Usually elevated, useful marker of disease activity, not usually checked
CBC: May show anemia (of chronic disease)
Liver enzymes: ALP, GGT may be increased. If AST/ALT increased, check CPK
Anti-cyclic citrullinated peptide (anti-CCP): Differentiate polymyalgic onset of RA from PMR
MRI/ultrasound examination of joints: May show proximal synovitis and/or bursitis
What are DDx for PMR?
Rheumatoid arthritis
Cervical myelopathy and bilateral radiculopathy
Inflammatory myositis: Weakness > pain, ^CPK/aldolase
Drug reactions: Eg. statins, colchicine
Bacterial endocarditis
Hypothyroid myopathy
Paraneoplastic syndromes
Fibromyalgia/depression
Late-onset spondyloarthropathy
RS3PE (RemittingSeronegativeSymmetricalSynovitis withPittingEdema)
Rotator cuff disease
Parkinson’s disease: Can sometimes present with pain
What are general principles for managing PMR?
Document symptoms and level of any disability at diagnosis
Consider giant cell arteritis (GCA) in all patients with polymyalgia rheumatica (PMR)
Advise patients with PMR to seek medical attention if they developed any symptoms of GCA
Monitor response to treatment by assessing changes in clinical features and inflammatory markers (ESR, CRP)
Manage any residual physical or psychosocial disability caused by the disease
What medication management should be done for PMR?
Prednisone is the drug of choice.
Treatment is initiated at a low dose, 10-20 mg a day (usually 15mg/day)
Response should be dramatic and quick (often patients will report complete improvement in 1-2 doses though can take a little longer) – if persistent achiness and/or inadequate response to >25mg prednisone, reevaluate diagnosis
No consensus for tapering, generally taper to 10 mg within a couple of months, then by 1mg a month or sometimes even more slowly – very steroid sensitive
Usually treated for more than a year
May need longer courses of treatment
Bisphosphonates, calcium, and vitamin D should be given to all PMR patients on chronic steroids based on guideline recommendations
Several drugs have been proposed as steroid sparing agents in PMR. These include:
Methotrexate (mixed data from RCTs but generally favorable with decreasing steroids and relapses)
IL-6 blockade (approved for GCA; promising data; need further trials)
TNF inhibitors (have failed to show benefit)
NSAIDs (not helpful)
Will PT help for managing PMR?
Physical therapy can be used as adjunctive tx but will not help alone
WHat is the prognosis for PMR?
PMR usually responds well to treatment with steroids resulting in remission in the majority of cases.
Relapse may occur, usually during taper below 10mg or within 2 years of stopping the steroids, but the condition remains steroid responsive.
Morbidity and mortality may occur as a result of immunosuppression or steroid side effects, and patients should be regularly monitored while on steroids.
What is Giant Cell Arteritis?
Most common primary systemic vasculitis in the U.S. affecting about 18 out of 100,000 people
Large vessel vasculitis
Often referred to as temporal arteritis as it frequently involves the temporal artery
Epidemiology is similar to that of PMR
Overlapping conditions: 20% of PMR with no signs of GCA have biopsy proven GCA and 40% of patients with GCA have symptoms of PMR
What are research classifications for diagnosis of Giant Cell Arteritis?
ACR classification criteria (1990):
Age at disease onset greater than 50 years
New headache or new type of localized pain in the head
Temporal artery abnormality: Tenderness/decreased pulsation, unrelated to atherosclerosis of carotids
Elevated ESR (> 50 mm/hour)
Abnormal artery biopsy showing vasculitis
3 or more must be present – can help inform diagnosis, but this is only technically for research purposes
Name different presentations for GCA
Headache weight loss fever fatigue visual symptoms anorexia jaw claudication PMR arthralgia unilateral vision loss bilateral vision loss vertigo diplopia
