360_Reiter.qxp 10/14/09 3:17 PM Page 360 Giant Cell Arteritis Misdiagnosed as Temporomandibular Disorder: A Case Report and Review of the Literature Shoshana Reiter, DMD Giant cell arteritis (GCA) is a systemic vasculitis involving medium Teacher and large-sized arteries, most commonly the extracranial branches Department of Oral Rehabilitation of the carotid artery. Early diagnosis and treatment are essential to avoid severe complications. This article reports on a GCA case Ephraim Winocur, DMD and discusses how the orofacial manifestations of GCA can lead to Lecturer misdiagnosis of GCA as temporomandibular disorder. GCA Department of Oral Rehabilitation should be included in the differential diagnosis of orofacial pain in Carole Goldsmith, DMD the elderly based on the knowledge of related signs and symptoms, Instructor mainly jaw claudication, hard end-feel limitation of range of Department of Oral Rehabilitation motion, and temporal headache. J OROFAC PAIN 2009;23:360–365 Alona Emodi-Perlman, DMD Key words: Giant cell arteritis, jaw claudication, Teacher temporomandibular disorders, trismus Department of Oral Rehabilitation Meir Gorsky, DMD Professor Department of Oral Pathology and Oral iant cell arteritis (GCA) is a systemic vasculitis involving Medicine the large and medium-sized vessels, particularly the extracranial branches of the carotid artery. It is more com- The Maurice and Gabriela Goldschleger G School of Dental Medicine mon in women (M:F ratio 2:5) and usually affects patients older 1 Tel Aviv University, Israel than 50 years with an increased risk with age. The highest preva- lence of GCA has been reported in Scandinavian populations and Correspondence to: in those with a strong Scandinavian ethnic background.2 Dr. Shoshana Reiter Permanent visual loss may result from GCA due to ischemia of Department of Oral Rehabilitation the optic nerve secondary to vascular occlusion. Therefore, GCA is The Maurice and Gabriela Goldschleger considered a medical emergency. The clinician’s awareness can School of Dental Medicine reduce the prevalence of visual loss associated with GCA (from Tel Aviv University 30% to 15%).3 Additional life-threatening conditions including Tel Aviv, Israel myocardial infarction, aneurysm of the aorta, infarction of the Fax: 972-3-6409250 intestine, renal insufficiency, pulmonary embolism, transient Email: [email protected] ischemic attacks, and stroke may also be related to GCA.4 Therefore, early diagnosis and appropriate management are essen- tial to avoid further complications.5 The most prevalent signs and symptoms of GCA include tempo- ral headache, jaw claudication, polymyalgia rheumatica (PMR) (neck, shoulder, hip pain, morning stiffness), constitutional syn- drome (asthenia, anorexia, and weight loss) and scalp tenderness.6,7 In addition to jaw claudication, orofacial manifestations of GCA include trismus, throat pain developing while chewing, changes in tongue sensation and tongue claudication, odontogenic pain, dys- phagia, dysarthria, submandibular mass, lip and chin numbness, macroglossia, glossitis, lip and tongue necrosis, and facial swelling.8 Jaw claudication is caused by arteritis of the maxillary artery which leads to an ischemia of the masticatory muscles. Approximately 40% of patients with GCA have jaw claudica- tion,8,9 but its prevalence has been reported as high as 65%.10 360 Volume 23, Number 4, 2009 © 2009 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY. NO PART OF THIS ARTICLE MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER. 360_Reiter.qxp 10/14/09 3:17 PM Page 361 Reiter et al Jaw claudication is considered one of the predictors test revealed an elevated erythrocyte sedimentation for an increased risk of permanent vision loss5 and rate (ESR) of 70 mm/h. The differential diagnosis could also represent a manifestation of other dis- at that point included GCA due to the elevated eases, such as amyloidosis/multiple myeloma,11–13 ESR and temporal headaches, and a possibility of atherosclerosis of the main blood vessels,14,15 and osteoarthritis of the temporomandibular joint Wegener granulomatosis.16,17 Therefore, these dis- (TMJ) based on the limitation of mouth opening, eases should be included in the differential diagno- pain upon chewing, and ear/TMJ area which were sis when jaw claudication is suspected. included in the description of the location of the Limited range of motion (ROM) of the pain. However, she was discharged from the ER mandible associated with GCA is a less frequently with a final diagnosis of left TMJ osteoarthritis reported sign compared to jaw claudication.18–25 and was referred to an oral surgery department for In a retrospective study,24 6.8% of the patients further treatment. Three days later she consulted presented with jaw “trismus.” These patients pre- with her dentist who suspected a dental cause for sented with a more aggressive disease, a higher her pain and extracted her mandibular left first percentage of eye involvement, and a shorter time molar. Following the extraction, the patient required for diagnosis. The authors concluded that reported no relief of pain and therefore was the prevalence of trismus is likely underestimated referred the following day to the Orofacial Pain due to the physician’s lack of awareness of this Clinic for further evaluation and treatment 14 complaint that is easily confused with jaw claudi- days after the initial onset of her chief complaint. cation. In another study,25 36% of GCA patients Her medical history included high blood pres- reported limited ROM. However, there was no sure (which was controlled by medications), vita- association between the limitation of ROM and min B12 deficiency, and myocardial infarction severity of the disease, eye involvement and delay (MI) 17 years previously. The patient was taking in diagnosis. The need to become familiar with disothiazide (25 mg/day), aspirin (100 mg/day), trismus as a sign of GCA was emphasized.24, 25 and vitamin B12 injections once a month. The It must be pointed out that jaw claudication and patient denied any history of headaches, trauma to its associated hard end-feel limitation of ROM (no the head and neck area, or TMD. increase in maximum mouth opening when apply- ing mild steady downward pressure to the lower Pain Characteristics incisors) may lead the clinician to misdiagnose GCA as a temporomandibular disorder (TMD), The patient described the onset of the pain as sud- especially in the elderly.26,27 The purpose of this den and pointed at the left ear/left TMJ area and article was to present a case of GCA and to review left temple as the sites of pain. The pain was the orofacial manifestations of GCA which can described as pressing and pulsating in quality, con- lead to misdiagnosis of GCA as TMD. tinuous, and gradually worsening. On a 0 to 10 visual analog scale (VAS), pain intensity was graded between 7 and 8 with at least 3 daily Case Presentation episodes, lasting from several seconds to 90 min- utes, of stronger pain (grade 10). The severe pain History was accompanied by nausea, vomiting, and photo- phobia and could awaken the patient from sleep- A 67-year-old woman who was referred by her ing. Any attempt to chew, open the mouth, or lie dentist to an Orofacial Pain Clinic complained of down aggravated the pain. The pain was not left temporal headache, limitation of mouth open- aggravated by physical activity and no autonomic ing, and pain upon chewing over the previous 2 signs were apparent. The malaise, depression, and weeks. The pain was initially controlled with inability to chew resulted in a 5 kg weight loss for paracetamol and dipyrone. However, the pain pro- the patient within a period of 2 weeks. gressively worsened and the patient presented to the emergency room (ER) 5 days after the onset of Clinical Examination pain. Since a computer tomography (CT) brain scan was within normal limits and neurological No unusual findings were noted on extraoral and and ear, nose and throat (ENT) examinations were intraoral examinations or on panoramic radiogra- nondiagnostic, she was discharged. Five days later phy. The TMJ examination was based on the the patient returned to the ER and complained Research Diagnostic Criteria for TMD examina- that her headache had severely increased. A blood tion guidelines (RDC/TMD).28 The mandibular Journal of Orofacial Pain 361 © 2009 BY QUINTESSENCE PUBLISHING CO, INC. PRINTING OF THIS DOCUMENT IS RESTRICTED TO PERSONAL USE ONLY. NO PART OF THIS ARTICLE MAY BE REPRODUCED OR TRANSMITTED IN ANY FORM WITHOUT WRITTEN PERMISSION FROM THE PUBLISHER. 360_Reiter.qxp 10/14/09 3:17 PM Page 362 Reiter et al Fig 1 Biopsy of the left superficial temporal artery Fig 2 Another section of the same artery as in Fig 1 showing thickening of the intima layer, narrowing of the showing intima thickening, disruption of internal elastic artery lumen, and no disruption of the internal elastic lamina, most probably due to an artifact, and scattered lamina (elastin staining by resorcin fuchsin method, plasma cells in the periphery (see arrow) (hematoxylin original magnification ϫ40). and eosin stain, original magnification ϫ40). opening pattern was straight but unassisted open- of the left superficial temporal artery was within ing without pain was limited to 30 mm. Maximum normal limits. Blood tests, taken the same day, unassisted opening and maximum assisted opening showed an elevated ESR (80 mm/h) and C reactive were limited to 32 mm and were accompanied by protein (CRP) (3.5 mg/dL). pain at the left TMJ and left masseter areas. There were no joint sounds upon opening, lat- Therapy and Follow-up eral and protrusive mandibular movements. Right lateral excursion was 9 mm, accompanied by pain Prednisone (60 mg/day) was prescribed. The in the left masseter muscle. Left lateral excursion headache disappeared within 24 to 48 hours of was 7 mm, accompanied by pain in the left mas- commencing steroid treatment and the ESR was seter muscle.