temporal arteritis / giant cell arteritis ( gca ) is a systemic immune - mediated vasculitis first described by horton et al .
that affects medium - sized and large - sized arteries , which include main branches of the aorta , its primary and auxiliary arteries , superficial temporal , ophthalmic , posterior ciliary and vertebral arteries .
it is commonly seen in older women more than 50 years ( m : f ratio 2:5 ) .
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
can drastically reduce the prevalence of vision loss associated with gca ( giant cell arteritis)/temporal arteritis .
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 ) . to avoid further complications , early diagnosis and appropriate management are essential .
temporal arteritis has a wide spectrum of clinical manifestation related to both systemic inflammation and ischemia .
the most prevalent signs and symptoms include temporal headache , jaw claudication , pmr ( polymyalgia rheumatica ) , scalp tenderness , and constitutional syndrome ( asthenia , anorexia , with appetite loss ) .
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 .
the present article discusses a rare case of temporal arteritis , rheumatoid arthritis with extra cranial manifestation ( interstitial lung disease ) presenting as pulo - periodontol pathology .
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 .
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 ] .
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 patient also complained of cough , breathlessness and weakness in the shoulder and legs .
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 .
orthopantomogram showing bone loss and furcation involvement in 16 and 17 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 ) .
ct scan of chest showing  diffuse ground glass opacity in bilateral lung fields with few areas of sparing  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 ] .
( 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
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 ) .
ct scan of chest showing  diffuse ground glass opacity in bilateral lung fields with few areas of sparing 
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 ] .
( 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
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 .
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
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 .
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 . 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 .
hence , common signs and symptoms of temporal arteritis should be differentiated from headache , ent pathology , and dental pathology . in 40 to 50% of cases ,
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 .
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 .
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 .
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 . in 1990 ,
the american college of rheumatology ( acr ) developed a set of criteria that have been used to diagnose gca / temporal arteritis .
the guidelines given by american college of rheumatology in 1990 are useful to diagnose temporal arteritis even today [ table 1 ] .
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 .
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 .
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 .
several studies have found that the sensitivity of crp alone is about 98% or higher for active temporal arteritis .
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 .
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 .
apart from the routine findings in a temporal arteritis case , and investigations showed elevated esr and crp levels .
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 .
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 ) . 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 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 . 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 , infliximab , and low - dose aspirin .
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 .
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 .
 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
CASE REPORT
Investigations
Histopathology
TREATMENT
DISCUSSION