Beth Uffner, an avid tennis player, developed an aching pain first in her legs, then in her shoulders that got progressively worse until she lacked the strength to get out of a chair unaided, let alone play tennis. Faith Sullivan, a novelist, developed a headache so devastating that she could hardly read and had to take a fistful of aspirin to get through a scheduled talk about her books.
Both women were in their mid-70s when they were seized by different but related inflammatory autoimmune conditions — polymyalgia rheumatica in Ms. Uffner, temporal arteritis in Ms. Sullivan. Both disorders can sometimes afflict the same person. And while both are life-inhibiting, giant-cell arteritis, as temporal arteritis is also called, is especially dangerous. A delay in treating the cause of this viselike head pain can result in irreversible vision loss, a stroke or even death.
Prompt and proper treatment with a corticosteroid like prednisone can quickly relieve symptoms of both conditions. But the therapy must be carefully managed by a knowledgeable physician — in most cases, a rheumatologist — to minimize side effects and produce a lasting remission.
As I learned from speaking with both women, it is important for people with symptoms suggestive of these disorders to resist the tendency to assume they’re nothing serious — that they will soon resolve on their own with an over-the-counter remedy to counter the pain. As a septuagenarian athlete, Ms. Uffner, a New Yorker, was used to having aches and pains. She self-treated with lots of ibuprofen for several months until this nonprescription remedy was no longer enough to get her through a normal day.
And as Ms. Sullivan, a Minneapolitan, now knows and cautions others, anyone who develops an unusual, severe headache should see a doctor without delay. As she described her symptom, “It was like an iron helmet on my head that kept getting tighter and tighter.”
Eventually, both women consulted their primary care doctors, who referred them to rheumatologists. Yet, as sometimes happens, both specialists made mistakes, resulting in a delay of effective treatment. Ms. Uffner was told that her symptoms were probably caused by osteoarthritis, a common condition among older adults that she’d had for years. Why, she wondered, would it have suddenly become so debilitating?
She then saw another more experienced rheumatologist, who suspected and soon diagnosed polymyalgia rheumatica and prescribed prednisone. Now, about three months later and on a lowered dose of prednisone, she’s back on the tennis court, though still not as energetic as she might be. With gradually decreasing doses, she said she expects all symptoms of this disorder to be gone, hopefully without further treatment, by this time next year.
And while the first rheumatologist who examined Ms. Sullivan correctly diagnosed temporal arteritis as the cause of her crippling head pain, the doctor failed to treat it with an adequate dose of prednisone. After several relapses of the excruciating pain when the drug dose was reduced, she saw another rheumatologist who treated the problem correctly, with a starting dose twice as high. But by then it took five years of very gradually reduced doses for the condition to finally resolve.
According to the American College of Rheumatology, about 15 percent of people with polymyalgia rheumatica will also develop temporal arteritis, and half of people with temporal arteritis will also have polymyalgia rheumatica, so anyone with either of these conditions should be alert to the symptoms of the other.
Although there is no cure for either condition, the good news is that both disorders respond rapidly to prednisone or a related drug and, when adequately treated, will eventually go away. In fact, prednisone is so effective that if symptoms are not relieved within a few days when a proper dose is taken, chances are the diagnosis is incorrect.
Polymyalgia rheumatica, or PMR, more commonly afflicts women — most often Caucasians. The average age of onset is 70 and it rarely occurs in people younger than 50. No one knows why a person’s immune system suddenly attacks the body’s own connective tissue, although genetics are believed to play a role and an infection may be the precipitating factor.
It’s a tricky diagnosis because the symptoms, which may start gradually or suddenly, can resemble a lot of different conditions. As Ms. Uffner experienced, PMR typically starts with pain and stiffness in the hips, thighs, neck, upper arms and shoulders that is most severe in the morning or following other prolonged periods of inactivity. Weakness and fatigue soon follow, sometimes accompanied by a mild fever, poor appetite and weight loss.
Making the correct diagnosis is a process of elimination. Many blood tests are done to rule out conditions like rheumatoid arthritis, lupus, Lyme disease, vasculitis, various muscle and infectious diseases, hormone abnormalities and even cancer.
Once PMR is diagnosed, it’s also important for patients to be checked for signs of temporal arteritis. If someone has symptoms suggestive of arteritis, a biopsy of the temporal artery on the side of the head may be needed to confirm the diagnosis.
The symptoms of temporal arteritis are not nearly as confusing. They result from inflammation of the temporal arteries that feed blood to the head, and that’s nearly always where it hurts the most. Patients may also have scalp tenderness, vision changes or soreness of the face or jaw, especially when chewing. Other possible symptoms include feeling sick, fever, fatigue and achy arms or legs. The most serious complication is a stroke, which afflicts about one person in 20 who has temporal arteritis.
Steroids like prednisone work by curbing the function of inflammatory cells that are the hallmarks of both disorders. But the medication has its own challenges. While it can give patients an energy boost, it also stimulates the appetite and fosters water retention, causing facial and abdominal bloating and weight gain.
More serious, however, is immune suppression, which is the secret to steroids’ therapeutic success but also one of their main risks: They can increase a patient’s susceptibility to infection. Patients on long-term steroids should be evaluated frequently for possible side effects. Blood tests can often detect risks before they cause any noticeable symptoms. Among needed checks are those for heart and lung function and blood sugar, as well as certain nutrients. Because steroids can weaken bones, patients are usually advised to take supplements of calcium and vitamin D and have their bone density measured periodically.
Finally, for people who cannot take steroids or who don’t benefit adequately from them, there are other drugs, including tocilizumab, that could be used instead.