Syndrome characterized by aching and stiffness of the neck, shoulders, and pelvic muscles.
it is characterized by pain and morning stiffness of the shoulder and pelvic girdles, with significant effect on quality of life and function.
PMR as an agent sex suggested prevalence rate in the US estimated at 701 per hundred thousand population aged at least 50 years.
Affects approximately 0.7% of patients older than 50 years, may occur alone or in conjunction with a giant cell arteritis.
Etiology unknown.
A diagnosis of exclusion.
A chronic inflammatory disease without universally except diagnostic criteria.
Proximal muscle group pain.
Muscle biopsy normal.
Muscle strength normal as is EMG.
Similar to giant cell arthritis, but is probably a distinct entity.
Giant cell arteritis and PMR almost exclusively affect individuals aged at least 50 years and frequently have overlapping symptoms, such as fever, fatigue, weight loss, depression, and night sweats, and elevation of inflammatory markers such as erythrocyte sedimentation rate and c reactive protein.
Prevalence 1 in 200 people over the age of 50 years.
Usually affects people older than 50 years of age.
Bilateral shoulder aching.
Bilateral subdeltoid bursitis is present on ultrasound in 69% of patients with PMR.
Whites affected more commonly than other races.
Females:males, 2:1.
Symptoms typically worse in the morning.
Symptoms are symmetrical.
Stiffness more pronounced after prolonged inactivity.
May have low grade fever, weight loss, fatigue, depression and rapid onset of symptoms.
Examination reveals no muscle atrophy or muscular tenderness.
Increased risk with age over 50 years, presence of giant cell arthritis.
ESR greater than 50 mm/h.
Anemia present in 50% of patients.
Chronic anemia screaming now or thrombocytosis are common
Low grade fever, fatigue and weight loss are frequently present.
PMR is included in the differential diagnosis of patients with acute onset of bilateral upper extremity pain, which is often worse with all the following rest.
DIAGNOSIS:
Diagnosis is based on presenting signs, symptoms, and laboratory results but there is no specific diagnostic test for PMR.
It is characterized by subacute or acute onset of aching pain and morning stiffness in the shoulders and neck.
Approximately 50 to 70% of patients with PMR have neck or hip girdle involvement, but peripheral joint symptoms are less frequent.
Elevation in acute phase reactants such as C reactive protein and erythrocyte sedimentation rate, and constitutional symptoms such as fever, weight loss, and fatigue are also characteristic.
CK is normal.
Mild liver test abnormalities may occur.
Not life threatening.
Treatment is with corticosteroids.
Starting dose of prednisone is usually 10 to 20 mg per day.
With initiation of corticosteroids symptoms usually resolve within a few days.
Requires treatment with steroids for 2-4 years.
Relapse common and the disease may reactivate with tapering of steroids.
Zero to 13% of patients may require higher initial doses of corticosteroids to control symptoms (Houston MC).
Methotrexate may be useful as a glucocorticoid agent with a dose of 10 mg per week.
Tocilizumab is considered in refractory disease.
Prophylaxis bisphosphonates, and calcium, and vitamin D supplementation or generally utilize to decrease corticosteroid related adverse effects.
More than half of patients with PR have a relapse doing tapering of glucocorticoid therapy.
Glucocorticoid associated adverse effects are reported in the range from 3.6-to 27% of patients in some series, and as high as 58 to 91% in other series.
It is suggested that interleukin-6blockade may clinically be clinically useful in the treatment of PR.
Sarilumab shows significant advocacy in achieving sustained remission in reduction in cumulative glucocorticoid dose in patients with a relapse of PR or during glucocorticoid tapering.
Most patients require only modest doses of corticosteroids, less than 25 mg a day: A rapid response establishes the diagnosis, and the need for higher doses suggested alternative diagnosis.
Prednisone is typically tapered over many months, and relapse occurs in more than half of patients.
Among patients with active polymyalgia rheumatica despite prednisone therapy, tocilizumab, compared with placebo, results in greater percentage of patients with a CRP protein level of less than 10 with reduced prednisone requirements at 24 weeks.
Patients with elevated sedimentation rate, CRP, and interleukin-6 levels at the time of diagnosis correlate with increased risk for relapse and for a higher corticosteroid dose requirement.
interleukin-6 has been implicated in the pathophysiology of polymyalgia rheumatica, because circulating elevated levels and increased tissue expression of IL-6 have been identified in patients.
Low ESR values, and higher hemoglobin levels are associated with better corticosteroid responses.
Women have more resistant disease, more relapses and a greater requirement for glucocorticoid drugs, and have more steroid related adverse effects, and require a longer duration of corticosteroid treatment than men.