A rare case of temporal arteritis with rheumatoid arthritis and interstitial lung disease mimicking pulpo-periodontal pathology.

A 75-year-old male patient was planned for dental treatment due to pain of suspected pulpo-periodontal origin in relation to right maxillary first molar. Careful evaluation revealed the pain to be non-odontogenic in nature and led to the diagnosis of temporal arteritis with rheumatoid arthritis along with interstitial lung disease (ILD). Characteristic findings of temporal arteritis include headache, jaw claudication, visual loss, and constitutional symptoms (malaise, fever, weight loss, loss of appetite). Temporal artery biopsy (TAB) remains the gold standard for diagnosis. Additional diagnostic tests include blood tests (ESR, CRP). This article reports and discusses how the orofacial manifestations can lead to misdiagnosis of temporal arteritis. Hence, temporal arteritis should be included in the differential diagnosis of orofacial pain in the elderly especially to prevent complications like vision loss.

INTRODUCTION

Temporal arteritis/Giant cell arteritis (GCA) is a systemic immune-mediated vasculitis first described by Horton et al.[1] that affects medium-sized and large-sized arteries,[23] which include main branches of the aorta, its primary and auxiliary arteries, superficial temporal, ophthalmic, posterior ciliary and vertebral arteries.[3] Cranial nerves (e.g. optic nerves) and posterior cerebral circulation may be affected. It is commonly seen in older women more than 50 years (M:F ratio 2:5).[4]

Vision loss due to ischemia of the optic nerves may result from temporal arteritis secondary to vascular occlusion. Since vision loss is permanent, temporal arteritis is considered a medical emergency. Hence, awareness on the part of clinician can drastically reduce the prevalence of vision loss associated with GCA (Giant cell arteritis)/temporal arteritis.[5] Additional life-threatening conditions like MI, aneurysm of the aorta, infarction of the intestine, renal insufficiency, pulmonary embolism, transient ischemic attacks and stroke may be related to temporal arteritis/GCA (Giant cell arteritis).[6] To avoid further complications, early diagnosis and appropriate management are essential.[7]

Temporal arteritis has a wide spectrum of clinical manifestation related to both systemic inflammation and ischemia.[8] The most prevalent signs and symptoms include temporal headache, jaw claudication, PMR (polymyalgia rheumatica), scalp tenderness, and constitutional syndrome (asthenia, anorexia, with appetite loss).[910]

Orofacial manifestations of temporal arteritis include trismus, throat pain during chewing, changes in tongue sensation, tongue claudication, odontongenic pain, dysphagia, dysarthria, submandibular mass, lip and chin numbness, macroglossia, glossitis, lip and tongue necrosis, and facial swelling.[1011]

The present article discusses a rare case of temporal arteritis, rheumatoid arthritis with extra cranial manifestation (interstitial lung disease) presenting as pulo-periodontol pathology.

CASE REPORT

A 75-year-old male patient came to Department of Periodontics, Army College of Dental Sciences with pain in the upper right quadrant since 2 weeks. The pain was continuous, sharp, and radiating to the right temple region. Intraoral examination revealed periodontal pockets in 16, 17 region and radiographic examination revealed a horizontal bone loss in 16, 17 region and furcation involvement of 16 with periapical radiolucency [Figure 1]. Tenderness on percussion was elicited and was diagnosed as perio-endo lesion. Endodontic treatment for 16 was decided as the first line of treatment followed by periodontal treatment. The patient gave a history of visit to ENT specialist 1 week prior with same complaint but ENT examination was non-diagnostic. Five days later, the pain increased drastically, wherein the patient described the onset of pain as sudden and pointed at the right ear/right TMJ area and right temple regions as the sites of pain. The pain was described as pressing, pulsating in quality, continuous and gradually worsening. The pain aggravated on chewing, opening the mouth, or lying down. The pain was not aggravated by physical activity. The patient also complained of cough, breathlessness and weakness in the shoulder and legs. Visible palpable swelling in the temporal region was noticed. The patient complained initially about blurring of vision and later partial loss of vision in the right eye. The patient was referred to an ophthalmologist who suspected temporal arteritis and was sent for temporal artery biopsy.

Figure 1

Orthopantomogram showing bone loss and furcation involvement in 16 and 17

Investigations

Since the patient also complained about cough, breathlessness, weakness in the shoulder and legs, blood tests like CBP (complete blood picture), ESR (erythrocyte sedimentation rate), SGPT (Alanine amino transferase), SGOT (Aspartate amino transferase), serum creatinine, plasma glucose, CRP (C-Reactive Protein), and thyroid function tests were done. The tests revealed elevated ESR 70 MM/HR, CRP- 3.6, SGOT levels - 172, Rheumatoid factor assay-119 IU/ml, and X-ray of knee revealed osteoarthritic changes. CT of lung and pulmonary function tests with spirometry were done which showed interstitial lung disease [Figure 2]. Diagnosis of temporal arteritis was confirmed along with rheumatoid arthritis and interstitial lung disease (ILD).

Figure 2

CT scan of chest showing “Diffuse ground glass opacity in bilateral lung fields with few areas of sparing”

Histopathology

Linear soft tissue specimen of the right temporal artery was taken for biopsy, which revealed thickening [Figure 3a] and presence of nodular projections in the intima with discontinuous endothelium [Figure 3b], variable myxoid and hyaline degeneration of the thickened wall in the sub intimal region [Figure 3c]. Inflammatory cells in sub intimal layer [Figure 3d]. However, granulomas and giant cells were not seen.

Figure 3

(a) H and E ×4 Showing cross section of temporal artery A - Showing the thickened artery wall B - showing the artery lumen (b) H and E ×10 A- nodular projections of tunica intima into the artery lumen B-inflammatory cells in tunica media (c) H and E ×40 A- hyaline degeneration in the subintimal layer B- subintimal layer showing inflammatory cells (d) H and E ×4 showing the longitudinal section of temporal artery A- thickening of intima, media, and adventitia B- with three layers tunica intima, media, and adventitia

TREATMENT

Since temporal arteritis is associated with serious systemic complications, management was done by a team of specialists comprising neurologist, ophthalmologist and rheumatologist. The goals of treatment were to halt progression of vision loss in the affected eye and prevent involvement of the other eye or other regions. Systemic corticosteroid therapy remains the mainstay of temporal arteritis treatment.[111213] An initial dose of 40-60 mg prednisone per day was started for the patient. The initial dose of prednisone was given for 4 weeks until all reversible signs and symptoms resolved and acute phase reactants CRP and ESR were normalized. The decision to taper the steroid dose was based on the regular assessment of clinical symptoms, signs, ESR and C-reactive protein levels. Dose tapering was done each week or every 2 weeks by a maximum of 10% of the total daily dose. The daily dose of prednisone was reduced by 5 mg every 2-4 weeks to about 25 mg/day. The range of taper was about 2.5 mg/day every 2-4 weeks until a dose of 10 mg/day was achieved. Tapering below 10 mg was done at intervals of 1-2 months by 2.5 mg/day.[14] Complications of long-term steroid therapy include osteoporosis, osteoporosis-related fractures, avascular necrosis of the hip, diabetes mellitus, infections, gastrointestinal bleeding and cataract. To prevent complications of steroid therapy, supplements of calcium (500 mg/day) and vitamin D (60,000 IU/week) were given. To prevent gastrointestinal complications, rabeprazole 20 mg was given once daily on empty stomach. To wean the patient from steroid therapy, methotrexate (2.5-5 mg/day) and aspirin (81 mg/day) were given. Bone mineral density study was performed for monitoring purpose. Scheduled visits were planned to check for relapse of temporal arteritis.

DISCUSSION

Patients with temporal arteritis may seek dental consultation because the first, and sometimes the only symptom is pain in the jaw or ear when chewing (“jaw claudication”). It can easily be mistaken for TMJ dysfunction, Myofascial pain syndrome (MFPS), acute alveolar abscess of the molar teeth and sinusitis and in the present case as endo-perio lesion. The pain described by the patient should be differentiated from odontongenic pain to avoid unnecessary dental treatment. Guttenberg et al.[15] described a report where inappropriate, ineffective endodontic surgery was performed in temporal arteritis case, which mimicked dental pain. The pain in temporal arteritis is localized to the anatomic area of the artery and radiated down to the ear and teeth, which is usually unilateral and will frequently cause scalp tenderness in the ipsilateral occipital area. It can resemble migraine due to its persistent throbbing quality over the temple area. But unlike migraine, it is characterized by burning pain as well. Hence, common signs and symptoms of temporal arteritis should be differentiated from headache, ENT pathology, and dental pathology.[14]

In 40 to 50% of cases, temporal arteritis is associated with polymyalgia rheumatica.[16] Polymyalgia rheumatica is a common inflammatory syndrome in elderly patient and has a diverse clinical profile that often overlaps with other rheumatic and inflammatory conditions and hence proper diagnosis should be done based on diagnostic criteria.[1718] Criteria for diagnosis of Polymyalgia Rheumatica includes age > 65 years, ESR over 40 mm/hr, bilateral upper arm tenderness, morning stiffness of more than 1 hour, onset of illness less than 2 weeks, and depression and/or weight loss.[17] This patient had both temporal arteritis and polymyalgia rheumatica. Over one third of patients with temporal arteritis have polymyalgia rheumatica at presentation. Some patients may have both polymyalgia rheumatica and temporal arteritis simultaneously; others evolve from one condition to the other.[19]

The patient was diagnosed initially with polymyalgia rheumatica but since rheumatoid factor assay was found to be 119 IU/ml, later the diagnosis was changed to rheumatoid arthritis.[20] In 1990, the American College of Rheumatology (ACR) developed a set of criteria that have been used to diagnose GCA/temporal arteritis.[21] The guidelines given by American College of Rheumatology in 1990 are useful to diagnose temporal arteritis even today [Table 1].[14] The presence of any three of the five criteria given by American College of Rheumatology permitted a diagnosis of temporal arteritis/Giant cell arteritis with a sensitivity of 93.5% and a specificity of 91.2% based on a population of patients (n = 807) with rheumatological disease.

Table 1

Classification criteria for giant cell arteritis

A case of temporal arteritis must be suspected in patients over the age of 50 years, presenting with pain of sudden onset. Other symptoms include fever, malaise, fatigue, and weight loss. If the patient reports transient vision loss, immediate referral to ophthalmologist is vital. There are no specific clinical symptoms or novel laboratory markers for the definite diagnosis of temporal arteritis/Giant cell arteritis; however, the combination of clinic, laboratory tests, and histopathology comprise the basis for diagnosis. ESR is the most widely used laboratory test to support clinical suspicion. Several studies have found that the sensitivity of CRP alone is about 98% or higher for active temporal arteritis.[222324]

Temporal artery biopsy (TAB) remains the gold standard test for diagnosis. But, it is also important to remember that a negative biopsy result may be found in up to 10 to 15% of all diagnosed cases.[25] The chief histopathological finding is a panarteritis consisting mostly of lymphocytes and macrophages. Thickening of intima and fragmentation of the internal elastic lamina are seen. Infiltration by mononuclear cells and concentration of multinucleated giant cells is around the inner half of the media, characteristically along the disrupted internal elastic lamina. It is important to remember that giant cells are present in about 50% of biopsy specimens and thus are not a necessary feature for histopathological confirmation of Giant cell arteritis.[2627]

Apart from the routine findings in a temporal arteritis case, and investigations showed elevated ESR and CRP levels. Other findings are thrombocytosis, anemia, elevation of cytokines like IL-6. There is an emerging evidence that IL-6 is a more sensitive indicator of disease activity than ESR or CRP; however, this test is not routinely available from most laboratories.[28] The less invasive investigative techniques which show promise for the diagnosis and monitoring of temporal arteritis/Giant cell arteritis (particularly large vessel disease) are ultrasonography, PET (Positron emission tomography), and MRI (Magnetic resonance imaging).[14] However, none of the above mentioned investigations is as valuable as temporal artery biopsy (TAB).

After diagnosis, prompt treatment was planned with a team comprising of ophthalmologist, neurologist, and rheumatologist. The corticosteroid therapy is the cornerstone for the treatment of Temporal arteritis. The present case is a complicated Giant cell arteritis (evolving visual loss), which required high dose of prednisone of 40-60 mg, calcium and vit D supplements. The initial dose of prednisone was given for 4 weeks until all reversible signs and symptoms have resolved and acute phase reactants CRP, ESR are normalized. Monitoring of the treatment was done by scheduled visits at 0, 1, 3, 6 weeks and then at months - 3, 6, 9, 12.[14] At each visit, full blood count, ESR/CRP was checked.

Because of long-term effects of steroid therapy which include diabetes, hypertension, secondary infections, and steroid related osteoporosis, prophylactic measures have to taken before starting the treatment. Periodic monitoring has to be done to check for initiation of steroid-related complications. To control steroid-related complications, steroid sparing agents have been studied in the treatment of temporal arteritis like azathioprine, methotrexate,[29] Infliximab,[3031] and low-dose aspirin.[832]

This case also presented interstitial lung disease, which is a rare extra cranial manifestation of temporal arteritis. Most patients with ILD (interstitial lung disease) present with nonspecific respiratory complaint such as cough or dyspnea. The investigations which help in diagnosis are chest X-RAY, pulmonary function test and high-resolution computed tomography.[33]

Temporal arteritis should be considered in differential diagnosis of pain of odontogenic origin, especially in an elderly patient with complaints of toothache, jaw claudication and throat pain during chewing. Therefore, a thorough case history that takes into account of patients’ age, all pain characteristics especially when pain radiates to the temple region, is mandatory for a correct differential diagnosis so as to prevent permanent complications like vision loss. Since temporal arteritis is mostly associated with polymyalgia rheumatica, which is easily confused with other rheumatological conditions and also has extracranial manifestations, proper understanding of different medical conditions by the dental professional is paramount so as to prevent unnecessary dental treatment.