ACR/EULAR Guidelines on PMR Management Finalized

Despite a lack of high-quality evidence about management of polymyalgia rheumatica (PMR), a joint effort by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR), with considerable input from the patient community, has achieved a multi-national consensus on the management of this baffling condition.

The final draft of the guidelines was prepared for presentation at the EULAR annual meeting in Paris this week. They are scheduled for later publication, following full review by both ACR and EULAR, in Annals of Rheumatic Diseases and Arthritis Care and Research and online on the ACR website.

PMR is the most common inflammatory rheumatic disease among the elderly, with an incidence of 700/100,000 in persons over the age of 50, noted Mayo Clinic rheumatologist Eric Matteson MD, co-principal investigator for the guidelines. Its chief symptoms, proximal pain and stiffness, significantly overlap many other conditions, among them malignancies, vasculitis, rheumatoid arthritis (RA), and systemic infections. As many as 30% of people initially diagnosed with PMR eventually turn out to have something else.

Although two British societies published PMR guidelines in 2010, and ACR and EULAR collaborated on classification criteria for PMR in 2012, ACR and EULAR felt a need for management guidance that would be appropriate to any health-care system. The resulting process relied on a systematic review of the literature (of more than 10,000 records, only 16 on interventions, 30 on prognosis, and 6 about both were deemed acceptable).

Of more than 10,000 records on PMR reviewed, only 52 were deemed of acceptable quality.

This was followed by surveys of rheumatologists and primary care physicians to judge practice standards.

High points of the new guidelines, meant for short- and long-term management in primary and specialty care, were described by the other member of the core management team, rheumatologist Bhaskar Dasgupta MD of Southend University Hospital in the UK:

1. Management should be directed toward excluding relevant mimicking conditions.

2. Before prescribing glucocorticoids, laboratory measures including rheumatoid factor (RF) and or ACPA, C-reactive protein (CRP) and/or erythrocyte sedimentation rate (ESR) should be assessed as a baseline to monitor treatment.

3. Initial evaluation and subsequent monitoring should include risk factors for steroid related side-effects, comorbidities, and other relevant medications and for relapse or prolonged therapy.

4. Steroid response should be assessed after 2-4 weeks for consideration of early referral to a specialist, in cases of partial or non-response.

5. Choice of initial glucocorticoid (GC) dose and subsequent tapering should be individualized.

6. In a personal communication about the guidelines, Dasgupta stressed that educating patients about the disease, its treatment and complications, and range of motion exercises for the shoulder and hips is "very important."

The guidelines recommend a starting dose of prednisone between 12.5 and 25 mg a day, according to Matteson, and methotrexate may be useful in early disease as a steroid-sparing agent. Biologics and NSAIDs are not endorsed, due to lack of evidence for their utility.

The quality of evidence is low even on the matters of initial GC doses and tapering, although PMR is the most common indication for their use, according to Christian Dejaco MD of the Medical University of Graz, Austria, who presented information about the literature review.

The data on prognosis are are contradictory, he told Rheumatology Network, but female gender, high initial ESR, and the presence of peripheral arthritis may portend a worse prognosis.

In general laboratory tests "tend to be unhelpful," according to Matteson, although a positive RF or ACPA might indicate RA, the presence of HLA-DRB1*04 may indicate underlying RA or giant cell arteritis, and an elevated CRP and or ESR are useful (but not specific) in separating PMR from non-inflammatory mimics. "I might add that typical ultrasound finding of shoulder bursitis and tendonitis particularly can be helpful in the diagnosis," he said.

The developers intend to update the guidelines within 3 years after their publication. As one purpose of the process is to highlight needs for new research, perhaps by then there will be good information to add.