Polymyalgia arteritis Arthur
DDS,a and Carl Runyon, LOS
and temporal DIMD,~ Los Angeles, Cali’
Polymyalgia rheumatica and temporal arteritis are separate but overlapping rheumatic diseases commonly seen among elderly persons. Polymyalgia rheumatica is characterized by upper body and trunk myalgias/arthralgias and an elevation in erythrocyte sedimentation rate. In about half of patients, temporal arteritis begins after polymyalgia rheumatica. Temporal arteritis is a systemic granulomatous disease that predominantly affects branches of the carotid artery. Claudication of the muscles of mastication and a painful burning tongue may develop during temporal arteritis or be the initial presenting symptoms. Recognition that these orofacial manifestations may be part of the disease process is mandatory because one third of patients with untreated temporal arteritis may go blind. (ORALSURC ORALMEDORALPATHOL 1990;69:317-21)
olymalgia rheumatica is a common rheumatic disease of elderly persons. ’ In a population aged 50 years or more, the incidence is 54 per 100,000, with women afflicted three times more often than men.2y 3 The onset of the disease may be insidious, but for many, it is acute, with patients able to give the specific date on which their illness began. There is aching pain, stiffness, and soreness in the proximal muscles of the hip and shoulder girdles, neck, and buttocks. The symptoms are generally symmetric, and patients may complain of an inability to lift their arms and difficulty in combing their hair, brushing their teeth, or dressing. Pain in the muscles of the trunk makes walking up stairs or getting up from a chair difficult. The symptoms are usually worse in the morning and after rest. Nocturnal discomfort interferes with sleep.4 A reactive depression often develops because of long-term pain, a lack of sleep, and a change in life-style. Constitutional symptoms include a lowgrade fever, night sweats, fatigue, malaise, anorexia, and weight loss.
aFormerly: Chief, Dental Service, Brentwood Division, West Los Angeles Veterans Administration Medical Center. Currently: Chief of Dental Service. VA Medical Center, Sepulveda, Calif. Associate Professor of Oral and Maxillofacial Surgery, School of Dentistry, University of California, Los Angeles. bChief Resident, Department of Oral and Maxillofacial Surgery, University of California, Los Angeles Medical Center. 7/13/11605
The most striking clinical laboratory finding is a very rapid erythrocyte sedimentation rate; it is almost always over 50 mm/hr (Westergren method) and often in the range of 80 to 120 mm/hr.5 A mild-to-moderate hypochromic or normochromic anemia is present in more than half of patients.6 Several other diseases (i.e., rheumatoid arthritis, polymyositis, systemic lupus erythematosus, Sj(igren syndrome, viral infection, and fibromyalgia) have a similar clinical appearance and must be ruled out. Treatment consists of low-dose steroids, usually prednisone, 10 to 15 mg/day. The sedimentation rate usually falls to normal levels within 4 days, but therapy must be continued for 4 to 6 months at the initial dose. The dose is then tapered to 5 mg/day, and medication is continued for an additional 3 to 6 months. Unfortunately, during this period of treatment, between 10% and 50% of patients have continued flare-ups and develop temporal arteritis,7-9 Temporal arteritis is a systemic granulomatous arteritis that predominantly affects branches of the carotid artery. It may develop after polymyalgia rheumatica or it may arise de novo. In a population aged 50 years or more, the incidence is 12 per 100,000, with women afflicted three times more often than men. There appears to be a higher incidence of the disease in more northern latitudes than in temperate zones. lo The cause and pathogenesis remain unknown, although there is a strong suspicion that an alteration in cell- or humoralmediated immunity will ultimately play a significant 317
role.’ ’ Reports of familial aggregation and a predominance in whites suggest a genetic predisposition.12 Temporal arteritis may begin with vague constitutional symptoms of fever, fatigue, malaise, anorexia, weight loss, and night sweats that mimic an occult malignancy, but the absence of generalized or localized lymphadenopathy and a diffuse aching of the neck, chest, and scalp muscles suggest temporal arteritis. Headache is the most common complaint and is present in as many as 90% of patients. It may be severe, unilateral, or bilateral and is often localized to the temporal areas along the route of the superficial temporal artery. Patients are often unable to sleep because of the intensity of the pain. Scalp tenderness occurs in most persons and is noted when they brush their hair or wear a hat. Ocular symptoms arise in about half of all patients. Intermittent diplopia may occur because of ischemia of the ocular muscles or an ischemic palsy of cranial nerves III, IV, and VI.13 Blindness occurs in one of three untreated patients. Arteritis of the short ciliary vessels leads to ischemia and infarction of the optic nerve. 14, I5 Complete visual loss is often preceded by a transient visual loss (amaurosis fugax) and is described by patients as a “skim” or “curtain over the eye.“16 Masticatory muscle pain and fatigue brought on by chewing and relieved by rest occurs in as many as half of patients. I7 Arteritis of the arterial blood supply to the temporalis and masseter muscles results in ischemia and claudication (pain). The blood supply to the temporalis muscle is the middle temporal branch of the superficial temporal artery and the anterior and posterior deep temporal branches of the internal maxillary artery. The blood supply to the masseter muscle is the transverse facial branch of the superficial temporal artery, the masseteric branch of the internal maxillary artery, and a muscular branch from the facial artery.‘* Mastication may also produce pain by irritating the superficial temporal artery. l 9 In some patients rapid protrusive movements of the tongue can bring about lingual pain, burning, and fatigue. 2o Lingual claudication is usually seen in association with a throbbing headache.2’ If the tongue also appears pale (blanched), dusk-blue, and is cold to the touch, this may portend an impending lingual infarction. 22 A recent major study has identified clinical manifestations of lingual arteritis in 25% of oatients with tm-qmra! arteritis. In fact, in this group of patients 15% had evidence of lingual infarction.23 Autopsy examination of patients with routine temporal arteritis showed that 90% had bilateral lingual arteritis.24
ORAL PATHOL March 1990
Other symptoms of temporal arteritis include dysphagia (because of ascending pharyngeal artery disease), facial paralysis, vertigo, hearing loss, cough, and hoarseness.25-29 If the intracranial vessels are affected, then a cerebrovascular accident, psychosis, or diabetes insipidus may occur.3o’ 3’ Physical examination showed that in approximately 70% of patients, the temporal artery is tender, thickened, nodular, and in advanced disease, pulseless. Occasionally there is hair loss and ischemic ulcerations of the scalp along the route of the temporal artery. Bruits in the carotid, subclavian, and brachial arteries are common and disappear once medical treatment is instituted.32 A mild anemia (normochromic or hypochromic and normocytic) refractory to iron therapy occurs in most patients. 33 Leukocytosis, thrombocytosis, and eosinophilia may be present. A markedly elevated erythrocyte sedimentation rate (Westergren method), frequently greater than 100 mm/hr, is usual. Rheumatoid factor, antinuclear antibodies, lupus erythematosus preparation, and serum complement levels are negative or normal. Mild elevations in alkaline phosphatase and aspartate transaminase occur in approximately one third to one half of patients and return to normal values once therapy has begun.34 Recently, elevation of the von Willebrand factor has been reported.35 Several other diseases have a similar clinical appearance and must be excluded. Polyarteritis nodosa, hypersensitivity vasculitis, arteriosclerotic disease, malignancy, infection, and other causes of fever, headache, blindness, and facial pain must be ruled out. A temporal artery biopsy is recommended for confirmation of the diagnosis because of the morbidity associated with high-dose, long-term steroid therapy especially in elderly persons. The specimen is obtained from the patient’s most symptomatic side by means of a local anesthetic. Microscopically in the early stages of the disease, there is segmental inflammation in the elastic lamina or adventitia with macrophages and lymphocytes seen. In advanced stages of the disease, necrosis of the arterial wall occurs, and granulomas containing multinucleated histiocytic and foreign body giant cells are noted. Thrombosis frequently occurs, which narrows the lumen to a small slit. Because blindness is sudden, unpredictable, and permanent, many practitioners institute steroid therapy before a confirmatory biopsy. Biopsy specimens are positive in 80% to 95% of patients with untreated temporal arteritis, but once steroid medications are started, there is an alteration in the inflammatory response, and only 60% of biopsy specimens are
positive after several days of treatment and less than 10% are positive after 1 week of treatment.36 Steroids are remarkably effective in the treatment of temporal arteritis and usually ameliorate the symptoms and lower the erythrocyte sedimentation rate within days. Treatment is begun with a moderZ%ly large dose of 40 to 60 mg of prednisone daily. The effective starting dose is continued until all reversible signs and symptoms are gone and the results of laboratory tests are normal (usually 2 to 4 weeks). The dosage is then slowly reduced to a dose that suppresses all symptoms and maintains a normal erythrocyte sedimentation rate. Once a minimum dose is established, it is maintained for 2 years. Approximately 15% of patients develop steroid toxicity during therapy. Complications include gastric hemorrhage, osteoporosis with vertebral compression fractures, cataracts, and diabetes mellitus. Close follow-up must be maintained for at least 2 years after steroids are discontinued because on rare occasions symptoms of temporal arteritis can recur after cessation of therapy. In most cases temporal arteritis is a self-limited disease. The statistical survival rate for patients with temporal arteritis is not different from that of the general population.37 CASE
A 70-year-old white woman came to the Medical Center with a complaint of pain on the right side of her face for 1 month. Pain localized to the masseter and temporalis muscles was brought on by chewing and relieved by rest. She claimed that this was the first occurrence of the problem. She also complained of an intermittent but severe right temporal headache that developed 2 weeks after the jaw and facial pain and prevented her from sleeping most nights. The patient, a retired office worker, related that 9 months earlier she had been diagnosed as suffering from polymalgia. Her signs and symptoms at that time were shoulder and pelvic girdle arthralgia, myalgia, an elevated erythrocyte sedimentation rate of 70 mm/hr (Westergren method), and a low-grade fever of up to 38.5” C. Because of a lack of appetite, she had lost 11 pounds in the 2 months preceding her diagnosis. She initially received 15 mg of prednisone a day, with resolution of all signs and symptoms by the fourth day. She continued to take that dose for 1 month, after which the steroids were tapered to a maintenance dose of 5 mg/day. The patient’s medical history included a right radical mastectomy for breast cancer 8 years before the onset of polymyalgia. At a recent follow-up examination performed by her surgeon, she was informed that she remained free of the disease. Six months ago the patient was seen by her opthalmologist and was diagnosed as having early cataract formation in her left eye that was not yet amenable to surgery. Bilateral myopia was noted, and corrective lenses were prescribed.
The head and neck examination disclosed exquisite scalp tenderness and moderate tenderness of the masseter and temporalis muscles. Cervical lymphadenopathy was absent. Intraoral examination showed evidence of recent and extensive dental reconstruction and early generalized periodontal disease. The patients oral hygiene was fair. Results of an occlusal analysis and temporomandibular joint examination were normal. The patient was requested to repeatedly open and close her jaws, but within 40 seconds she had to stop because of a sharp burning sensation in both the right and left masseter and temporalis muscles. After resting for 2Y2minutes the pain abated. The patient was then requested to thrust her tongue forward and to the right and left. She was able to continue the exercise for 90 seconds, but then stopped because of lingual pain and fatigue. Throughout these maneuvers, the tongue remained normal in color and temperature. Results of periapical radiographs and a panoramic radiograph were normal. Radiographs of the temporomandibular joint and maxillary sinuses were also normal. The clinical impression was an onset of temporal arteritis with orofacial manifestations, which were superimposed on a prexisting case of polymyalgia rheumatica. The patient was referred to her physician, who obtained the following laboratory clinical data: erythrocyte sedimentation rate was elevated to 97 mm/hr (Westgren: normal,