Polymyalgia Rheumatica Treatment & Management

Updated: Nov 25, 2018
  • Author: Ehab R Saad, MD, MA, FACP, FASN; Chief Editor: Herbert S Diamond, MD  more...
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Treatment

Approach Considerations

Polymyalgia rheumatica (PMR) is a chronic, self-limited disorder. Therapy is based on empiric experiences because few randomized clinical trials are available to guide treatment decisions. The therapeutic goals are to control painful myalgia, to improve muscle stiffness, and to resolve constitutional features of the disease.

Corticosteroids (ie, prednisone) are considered the treatment of choice because they often cause complete or near-complete symptom resolution and reduction of the erythrocyte sedimentation rate (ESR) to normal. However, no definite evidence demonstrates that corticosteroids (or any other therapy) alter the natural history of PMR. Before the corticosteroid era, patients with PMR occasionally experienced spontaneous improvements, and musculoskeletal symptoms were treated with nonsteroidal anti-inflammatory drugs (NSAIDs). The low-dose corticosteroids used in PMR are almost certainly ineffective in the prevention of vasculitis progression.

Joint guidelines from the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) conditionally recommend starting corticosteroid therapy with 12.5-25 mg/day of prednisone or the equivalent. [23] A systematic examination of the peer-reviewed literature, which included 30 studies, found that remission of PMR seemed to be achieved for most patients with a starting dose of prednisone at 15 mg/day. A slow tapering of the prednisone, less than 1 mg/month, was associated with fewer relapses. Once prednisone is tapered to 10 mg/day, a slow taper by 1 mg every 2 months until treatment discontinuation was associated with optimal control of disease activity. [32]

Nevertheless, controversy remains regarding dose and duration of treatment. Dose depends on the patient's weight and severity of symptoms. Expect prompt relief of symptoms within 24-72 hours. Corticosteroid dose should be increased if symptoms are not well controlled within 1 week, and a diagnosis of giant cell arteritis may need to be pursued, especially if prednisone 20 mg/d does not control symptoms. In contrast to other rheumatic diseases, alternate-day administration of corticosteroids in PMR has largely been unsuccessful.

Tapering should be guided by clinical response to include decreased pain and stiffness, decreased morning stiffness, and decreased shoulder pain/limitation on clinical examination. [33, 34] Normalization of inflammatory markers is also helpful but should not be used as a guideline for decreasing or stopping treatment. Patient-reported outcomes including global pain, hip pain, morning stiffness, physical function, mental function, as well as inflammatory markers have been reported as the best measures of disease activity and response to treatment.

If not contraindicated, NSAIDs may provide supplemental pain relief. However, per a study of 232 patients with PMR by Gabriel and colleagues, NSAIDs are associated with considerable drug-related morbidity and thus should be used with caution. [35]

Corticosteroid-sparing agents are sometimes considered in patients with PMR to reduce corticosteroid-related adverse effects, especially in certain patient populations such as diabetic patients or in those who develop osteonecrosis.

Methotrexate has been investigated in three randomized studies in newly diagnosed PMR. One study used methotrexate at 7.5 mg/week plus 20 mg/day of prednisone and found no benefit in outcomes after 2 years of follow-up. [36] Another study used oral and intramuscular methotrexate at a higher dose of 10 mg/week added to the prednisone regimen versus prednisone regimen alone. Overall, the patients receiving methotrexate 10 mg/week plus prednisone experienced corticosteroid-sparing effects. [37, 38]  A retrospective study of methotrexate in 100 patients with relapsed or steroid-resistant PMR reported benefit with doses of up to 20 mg/day. [39]

Limited but increasing data on tocilizumab, an interleukin-6 receptor antagonist, suggest that this agent is effective, safe, and well-tolerated in patients with PMR and has a robust steroid-sparing effect. [40] Early evidence also supports tocilizumab monotherapy as first-line treatment for PMR, instead of steroids. [41]

Tumor necrosis factor alpha (TNF-α) inhibitors have also been investigated as corticosteroid-sparing agents in PMR. A randomized study with infliximab revealed no benefit. [42] The only randomized trial using azathioprine (150 mg/d) during the maintenance phase of PMR showed a high frequency of adverse drug effects and a high number of patient withdrawals from the study, although a lower cumulative dose of corticosteroid at 52 weeks. At this time, the small number of completers and the high number of giant cell arteritis patients in the study make the study results difficult to interpret. [43] EULAR/ACR guidelines strongly recommend against the use of TNF-α inhibitors in PMR. [23]

Symptomatic palliation of pain with analgesic therapy alone with close monitoring may be preferable in patients with intolerable adverse effects from corticosteroids (eg, uncontrolled diabetes mellitus, severe symptomatic osteoporosis, psychosis).

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Diet and Activity

Calcium and vitamin D supplementation should be initiated in all patients with PMR who are starting corticosteroid therapy.

Generally, activity restriction is unnecessary. Physical therapy is recommended for those with difficulty achieving good mobility despite adequate medical therapy.

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Consultations and Long-Term Monitoring

Diagnosis and treatment involve the primary care physician and rheumatologist. In coordination with the primary care physician, the rheumatologist plays an important role in the diagnosis, treatment, and follow-up care. Ophthalmologists, pathologists, and surgeons may be consulted on an as-needed basis should concern arise about the development of giant cell arteritis.

Involvement of primary care providers is imperative, to assist with the management of comorbidities such as prophylaxis for cardiovascular disease, glucocorticoid-induced hyperglycemia, and osteoporosis. Appropriate immunizations should be administered, ideally before corticosteroid therapy is initiated.

PMR is typically treated in an outpatient setting. Determination of prognosis and of duration of treatment remain empiric and patients often need careful supervision.

Patients receiving steroids should have monthly follow-up, with regular monitoring of inflammatory markers. An isolated increase of ESR without symptoms during the course of treatment is not a valid reason to increase corticosteroid dose; however, a temporary delay in dosage reduction may be necessary. After steroid tapering, follow-up can be performed quarterly.

The risk of vertebral fractures is five times greater in women with PMR. A baseline bone mineral density study (eg, dual-energy x-ray absorptiometry [DEXA] scan) is recommended at the onset of treatment. As most patients require corticosteroids for at least 1-2 years, bisphosphonate therapy is recommended to prevent corticosteroid-induced osteoporosis. Interestingly, in an Italian, study, PMR patients who were treated with bisphosphonates were more likely to be able to discontine corticosteroids. [44]

Because relapses are more likely to occur during the initial 18 months of therapy and within 1 year of corticosteroid withdrawal, with a frequency of approximately 50%, all patients should be monitored for symptom of recurrence throughout corticosteroid tapering and for 12 months after cessation of therapy.

Relapses usually occur when the dose of prednisone is less than 5.0-7.5 mg/day or after therapy has been discontinued. If symptoms recur, the corticosteroid dose should be increased to the dose that previously controlled the symptoms. Recurrences of the disease more than 1 year after discontinuation of corticosteroid therapy has been reported. [45]

Approximately 50-75% of patients can discontinue corticosteroid therapy after 2 years of treatment. However, some patients may require low doses of corticosteroids for several years. In a population-based study from the United Kingdom, the median duration of  continuous corticosteroid treatment was 15.8 months, but around 25% of patients received more than 4 years in total of corticosteroid therapy. [16]

Patients with PMR should be monitored regularly and carefully for symptoms and signs suggestive of GCA development.

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