GRAND ROUNDS AT THE JOHNS CLINICIAN’S CORNER HOPKINS BAYVIEW MEDICAL CENTER Temporal Arteritis A Cough, Toothache, and Tongue Infarction David B. Hellmann, MD Temporal arteritis, the most common form of systemic vasculitis in adults, CASE PRESENTATION is a panarteritis that chiefly involves the extracranial branches of the carotid DR HELLMANN: My patient, Professor R, who is a 79-year-old woman, was artery. The condition is illustrated in this article by the case of a 79-year-old well until she developed a disorder that woman with a dry cough, toothache, tongue infarction, and vision loss. The eventually produced blindness in her mean age of onset is 72 years and the disease rarely occurs in persons younger right eye. Although most of the com- than 50 years. The most common presenting manifestations are headache, mon presenting features of temporal ar- jaw claudication, polymyalgia rheumatica, and visual symptoms. Eighty- teritis (TA) are familiar to general in- nine percent of patients have an erythrocyte sedimentation rate greater than ternists and family physicians, the many 50 mm/h. However, about 40% of patients present with atypical manifes- disguises of this condition may chal- lenge the diagnostic skills of any expe- tations, including fever of unknown origin, respiratory tract symptoms (es- rienced physician. Learning to see pecially dry cough), and large artery involvement. Familiarity with such un- through these disguises is crucial to usual manifestations of temporal arteritis facilitates early diagnosis and early diagnosis and the prevention of treatment, thereby reducing the risk of vision loss. visual loss. Professor R, please tell us JAMA. 2002;287:2996-3000 www.jama.com how your illness began. PROFESSOR R: All my troubles be- PROFESSOR R: No, it was dry. I saw a and tongue pain, I could not eat very well gan the month my husband and I were physician who gave me some pills but the and lost 20 pounds during the week we moving from our home into a retire- cough persisted. Two months later I con- were away. When we returned from Ha- ment community. Before that, I was tinued to cough and felt exhausted. Since waii, my first visit was to the dentist. He completely healthy, playing tennis, it was winter, we thought that if we got found nothing wrong with my teeth. It swimming, and hiking. I am now a pro- away for a week to Hawaii I would get was actually an ophthalmologist who fessor emeritus and still perform re- better. The afternoon before we left for first suspected the correct diagnosis be- search on iconography of libraries in the Hawaii I awoke from a nap and could not cause of my blindness and other symp- 17th and 18th centuries. It keeps me see out of my right eye. It was all black. toms; the eye doctor tested my sedimen- interested, traveling, and visiting li- I could not reach a physician so I told tation rate and then immediately braries in Europe. My husband and I my husband, “Let’s just go. By the time admitted me to the hospital. have been doing all sorts of exciting we get back I’m sure I’ll be seeing fine DR HELLMANN: That is right, the things and I have never been in a hos- again.” And we went on our trip. erythrocyte sedimentation rate (ESR) pital except for a broken arm. The DR HELLMANN: Did your vision get was 115 mm/h. Results of the other tests month we sold our house I was under better? performed at that time, including a a great deal of stress. Moving from a PROFESSOR R: No, it never did. complete blood cell count, serum chem- house into a 2-bedroom apartment, I DR HELLMANN: Did you experi- istries, and a chest radiograph, were had to give up a lot, especially my own ence a headache or any other symp- normal. She was treated with intrave- library of 2000 books. So, I was so tired toms, aside from the fatigue, cough, and that I began to take naps for the first Author Affiliation: Johns Hopkins University School visual loss? of Medicine, Department of Medicine, Johns Hop- time. I thought the tiredness would go PROFESSOR R: I never had a head- kins Bayview Medical Center, Baltimore, Md. away but after 2 to 3 weeks I also de- ache. But after I started coughing and be- Corresponding Author and Reprints: David B. Hell- mann, MD, Johns Hopkins University School of Medi- veloped a cough. fore I had the eye problem, I did de- cine, Department of Medicine, Johns Hopkins Bay- DR HELLMANN: Did you bring up velop a toothache. I could not quite view Medical Center, 4940 Eastern Ave, Baltimore, MD anything when you coughed? 21224 (e-mail: [email protected]). determine which tooth or teeth hurt. My Grand Rounds at The Johns Hopkins Medical Insti- mouth just hurt all over. Then I devel- tutions Section Editors: David B. Hellmann, MD, D. oped a burning sensation on the left side William Schlott, MD, Stephen D. Sisson, MD, The Johns See also Patient Page. Hopkins Hospital, Baltimore, Md; David S. Cooper, MD, of my tongue. Between my toothache Contributing Editor, JAMA. 2996 JAMA, June 12, 2002—Vol 287, No. 22 (Reprinted) ©2002 American Medical Association. All rights reserved. TEMPORAL ARTERITIS nous methylprednisolone in high doses the eighth decade.1,3 The average age of Table. Classic Symptoms and Findings and underwent a right temporal artery onset of TA is 72 years. Perhaps as a re- in Temporal Arteritis* biopsy. The biopsy showed granulo- sult of the aging population in this coun- Frequency, % matous inflammation with multinucle- try, greater recognition of the disease on Symptoms ated giant cells, rupture of the internal the part of physicians, or both, the inci- Headache 77 Jaw claudication 51 elastic lamina, and luminal narrow- dence of TA has been rising in some Constitutional symptoms 48 ing. These findings were diagnostic of populations.1 Temporal arteritis has been Polymyalgia rheumatica 34 temporal arteritis. One day after the bi- reported in all groups, but appears es- Visual symptoms 29 Findings opsy, Professor R was discharged tak- pecially common in people of Scandi- Fever 26 ing prednisone, 60 mg/d. navian or Northern European heri- Abnormal temporal artery 53 1,2 Erythrocyte 94 I saw Professor R for the first time 1 tage. Certain genes (ie, HLA-DR4 sedimentation week later. Her vital signs were normal. haplotypes 0401 and 0404/8, which are rate Ͼ40 mm/h She had minimal light perception in the contained in the HLA-DRB1 locus) have *Data derived from Machado et al.5 right eye with a relative afferent pupil- been associated with an increased risk of lary defect. That is to say, her pupils con- developing TA.7 Although the cause of sense of discomfort in or around the jaw stricted less when I shone a light in the TA is unknown, the disease appears to that may be unrelated to chewing. One right eye than when I shone the light in be T-cell dependent and antigen driven.8 of my other patients had a diffuse man- the healthy left eye. The right optic disc dibular discomfort that she attributed to was pale. The right temporal artery was Classic Manifestations her face-lift, even though that surgery had surgically absent while the left—barely The classic manifestations of TA are taken place months earlier and had pulsatile—was hard and stiff. An ische- headache, jaw claudication, polymyal- healed without difficulty. A sense of den- mic ulcer the size of a jellybean was pre- gia rheumatica (PMR), and visual symp- tal discomfort, as described by Profes- sent along the left lateral surface of the toms (TABLE).4-6,9 Headache is the most sor R, is another common variant of jaw tongue. The peripheral pulses were pal- common feature, occurring eventually in claudication. I have also seen patients pable and symmetrical, and there were more than 70% of patients. Although the with TA present with pain in the sinus no bruits in the carotid, subclavian, ax- headache often causes a deep aching pain region or in the ear. Some were treated illary, abdominal aortic, or femoral ar- over the temporal area, the headache can with antibiotics for sinusitis or otitis de- tery regions. be extremely variable in location, inten- spite the absence of any physical find- sity, and quality. Quite often, the only ings to support those diagnoses. Given DISCUSSION distinctive feature of the headache is that the variability in presentation of the head- Professor R’s presentation is instruc- it is new. Even if the patient has experi- aches and jaw claudication associated tive because it emphasizes some of the enced migraines or tension headaches for with TA, the diagnosis should be con- unusual ways in which TA can pre- years, he/she will note that this head- sidered whenever anyone older than 50 sent. When it presents atypically, as it ache is different. Alternatively, the pa- years complains of persistent unex- did in Professor R, it may not be diag- tient may say, “I am 72 and have never plained pain above the neck. nosed before the development of blind- had headaches until now.” Some pa- Polymyalgia rheumatica is defined as ness, the most feared complication of tients develop inflammation of the oc- pain and stiffness in the shoulders, neck, TA. Knowing the disguises that TA can cipital artery, causing pain at the base of and hip girdle areas and is worse in the wear affords physicians the best chance the skull. These symptoms are easily mis- morning and improves as the day goes of diagnosing and treating TA before the taken for cervical spine disease.