Actress Barbara Billingsley recently passed away at the age of 94. The 1950′s TV star who played June Cleaver on Leave it to Beaver was the model of American Motherhood in that era, always wearing a dress, high heels, and pearls, even while housecleaning. Ms. Billingsley suffered from rheumatoid polymyalgia. Billingsley is also well known for her cameo appearance in the 1980′s comedy Airplane!, informing a frazzled flight attendant “,’” and proceeding to translate the dialect of an ailing passenger. Her Leave it to Beaver “children” Jerry Mathers (“Beaver”) and Tony Dow (“Wally”) told :
“Barbara was a patient adviser and teacher. She helped me along this challenging journey through life by showing me the importance of manners and respect for others.” (Jerry Mathers)
“She was as happy as a lark being recognized as America’s mom.” (Tony Dow)
When Patients Ask What Polymyalgia Rheumatica Is
Polymyalgia rheumatica (PMR), is an inflammatory condition of the muscles in the neck, shoulders, hips and thighs. The pain and stiffness usually occur in the morning or after a period of inactivity. It typically lasts longer than 30 minutes. For most people, the condition develops gradually. But for some people it can develop quickly — even as quickly as overnight. In addition to stiffness, symptoms may include fever, weakness and weight loss.
PMR sometimes occurs along with giant cell arteritis (GCA) (see below), an inflammatory condition that causes swelling of the arteries in the head. Symptoms include headaches and blurred vision.
The cause of PMR is not known. HLA-DR4 is found with increased frequency in PMR patients and also in patients with giant cell arteritis. Systemic monocyte activation is characteristic of both conditions. High levels of IL-2 are associated with PMR and high serum levels of IL-6 correlate with increased disease activity.
One hypothesis holds that in a genetically predisposed patient, an environmental factor, possibly a virus, causes monocyte activation, which helps determine the production of cytokines that induce manifestations characteristic of PMR and giant cell arteritis. The prevalence of antibodies to adenovirus and respiratory syncytial virus was reportedly higher in PMR patients.
Polymyalgia rheumatica usually resolves within several years. Corticosteroids in relatively low doses are the most effective treatment: 15-30 mg/day prednisone for a few weeks tapered by 5 mg every week or two to very low doses (2.5-5.0mg/day), while monitoring symptoms and, intermittently, the erythrocyte sedimentation rate (ESR).
The symptoms of polymyalgia rheumatica are quickly controlled by treatment with corticosteroids, but symptoms return if treatment is stopped too early. Corticosteroid treatment does not appear to influence the length of the disease.
When Patients Ask About Giant Cell Arteritis
Giant cell arteritis (GCA) causes inflamed arteries of the scalp, neck, and arms. The arteries most affected are those in the temples on either side of the head. Early symptoms of GCA may resemble flu symptoms such as fatigue, loss of appetite, and fever. Symptoms specifically related to the inflamed arteries of the head include headaches, pain and tenderness over the temples, double vision or visual loss, dizziness or problems with coordination and balance. Pain may also affect the jaw and tongue, especially when eating, and opening the mouth wide may become difficult.
Like PMR, GCA is treated with corticosteroids, however larger doses (60-80 mg/day prednisone) are required. For the best prognosis, GCA should be treated as early as possible to prevent permanent complications including blindness and stroke. Temporal artery biopsy should be performed no more than 1-2 weeks after starting prendisone.
How Are Polymyalgia Rheumatica and Giant Cell Arteritis Related?
It is unclear how or why PMR and GCA frequently occur together. PMR occurs in about 50 % of patients who have GCA, while approximately 15% of patients with PMR develop GCA. There may be a common genetic component between the two disorders. Some people with polymyalgia rheumatica also develop giant cell arteritis either simultaneously or after the musculoskeletal symptoms have disappeared. Other people with GCA also have PMR at some time while the arteries are inflamed
How Are These Conditions Diagnosed?
The diagnosis of PMR and GCA are made with a combination of:
• Medical history
• Symptoms
• Physical exam
• Blood tests including an ESR, C-reactive protein and Rheumatoid Factor
The ESR is usually elevated to over 50 mm/hr (compared to the normal rate of 25-30). Patients often have a normochromic normocytic anemia and slight elevations in alkaline phosphatase (a mild inflammatory reaction in the liver is not uncommon). Thyroid-stimulating hormone (TSH), serum protein electrophoresis and other laboratory tests are normal. Rheumatoid factor is normal or may be slightly elevated.
The gold standard to diagnose giant cell arteritis is a temporal artery biopsy. A positive histologic diagnosis demonstrates inflammation of the arterial wall with fragmentation and disruption of the internal elastic lamina. Multinucleated giant cells are found in less than half of the cases and are not specific for the disease.
For more information about Polymyalgia Rheumatica, click to go to the Resounding Health Casebook on the topic. Here is a on YouTube.