Internal Medicine Rheumatology

Temporal Arteritis

Temporal Arteritis
Palpating the temporal artery is one of the first steps in diagnosing temporal arteritis.

Introduction – Temporal Arteritis

Temporal arteritis (TA) is an inflammatory disease of the elderly, often affecting any segment of the temporal artery or its branches. However, the inflammation may be present in other extracerebral medium and large arteries and sometimes even in the distal branches of the aortic arch. The symptoms then arise from a different location, for example, the back of the head when the occipital artery is engaged or the tongue if the lingual artery is affected. A better, more comprehensive term is thus cranial arteritis. From the histopathological perspective, the condition can also be called giant cell arteritis.

TA does not occur in people younger than 50 years and the incidence rises with increasing age.

Temporal arteritis and polymyalgia rheumatica (PMR) sometimes occur together and it is well known that the diseases are associated with each other. In the total TA / PMR population, 38% have only TA symptoms, 45% PMR and 17% have mixed symptomatology. The reason for the link between the two diseases is not clear.


Symptoms – Temporal Arteritis

Symptoms common to polymyalgia rheumatica:

  • Subacute onset of inflammation-related general symptoms such as:
    – Fatigue
    – General malaise
    – Decreased appetite
    – Weight loss
    – Subfebrility

    Sometimes preceded by influenza-like symptoms

In cranial arteritis, the following ischemia-related symptoms can emerge:

  • Localized superficial headache, previously not experienced by the patient. Distribution depending on which vessel segment is involved: usually the temple but the scalp, hind head, cheek and area around the ear may also be involved.
  • Scalp tenderness
  • Jaw claudication
  • Sometimes visual impairment
  • Aortic arch symptoms in 10-15% of cases

Clinical Findings – Temporal Arteritis

  • Substantial superficial tenderness of the scalp.
  • Sometimes swollen, lumpy, tender temporal artery or any of its branches, often with weak pulses in the vessel in question.
  • Jaw claudication when circulation in the chewing muscles is involved.
  • Ischemic Vision impairment:
    • Amaurosis fugax or blurred vision with visual field loss ( a. Centralis retinae or its branches).
    • Ptosis (arterial supply to m. Orbicularis oculi).
    • Diplopia (arterial supply to the extraocular muscles).
    • Often normal ophthalmoscopy.
  • An unknown proportion of patients with temporal artery disease have evidence of aortitis seen in computed tomography. Studies have estimated the aortitis to be present in 10-20% of the cases.
  • Occasional cases of intracerebral arteritis with stroke are reported.

Differential Diagnoses

Diagnosis Excluded with
Pain/Referred pain from cervical spondylosisNeurological examination of the upper extremities
MigraineMedical history
Arthritis or malocclusion of the temporomandibular jointExamination of the jaw joint, occlusal surfaces of teeth
Tension headachesHistory, palpate shoulder-neck musculature, scalloped tongue
Trigeminal neuralgia Cranial Nerve Examination
Postherpetic neuralgia Herpes zoster in Medical History
Intraocular hypertension/glaucoma Field of view, ocular pressure measurement, retinal examination
Palpable arteriosclerosis in temporal artery with SR increase of other causeHistory/physical examination – alternative explanations for SR increase, general arteriosclerosis?
Part of general vasculitisMedical History, general physical examination. Cutaneous vasculitis?  Nephritis?  Positive ANCA?

Diagnostics – Temporal Arteritis

  • There is no specific blood test
     
  • Medical history and physical examination as above, palpate the temporal and radial arteries and auscultate the carotids. Blood pressure in both arms.
     
  • SR (or CRP). Please note that SR can be normal at the debut and rise within a few weeks. 10% of TA patients are reported to have normal SR. In the case of clinical relapse, up to 50% have normal SR.
     
  • Biopsy of the temporal artery: the specimen should be at least 3½ cm long and taken from an aching portion of the vessel, or where the vessel lacks a pulse. The shorter the biopsy segment, the less diagnostic significance a negative outcome of the biopsy has.
     
  • B-glucose or P-glucose to exclude diabetes before steroid therapy.
     
  • Chest X-ray – possibility of TB (must be considered before long-term steroid therapy).
     
  • In case of suspected large vessel involvement, computer tomography, MRI or PET scan may be required.

Otherwise, the diagnostic procedures are guided by the medical history.

Autoantibody analysis has no value if there is no clinical suspicion of SLE or systemic vasculitis.

The risk of blindness is greatest initially in the process, which is why a biopsy must not delay further treatment. Seek biopsy on the same day or at the latest a day after the decision to perform the procedure has been made.

Clinical findingMeasure
Typical symptoms with no visual impactDo elective biopsy, preferably before steroid therapy
Visual impact, typical symptomsImmediately treat, elective biopsy later
Vague suspicion in major SR diagnosticsElective biopsy
Atypical vision symptom and/or clinical findingAcute biopsy

It has been discussed whether steroid therapy started before biopsy affects the outcome. The literature is contradictory in this area.

For typical symptoms and findings, patients can be cared for in primary care. In case of slight suspicion of visual symptoms caused by TA, the patient should be referred urgently to the hospital.


Treatment – Temporal Arteritis

Glucocorticoids are the only therapy initially. See details below for use of tocilizumab (Roactemra)

The treatment recommendations regarding the dosage of prednisolone vary within the country and scientific support exists for several different approaches. In the first place, follow the recommendations at a local rheumatology clinic.


The following schedule can serve as a rule of thumb:

  • In visual symptoms
    Pulse infusion methylprednisolone (Solu-Medrol) 500 or 1000 mg/day iv for three days, then Prednisolone po 60 mg/day until symptom remission or for at least one week, after which the daily dose is gradually lowered at the same rate as described below under the heading “tapering”. Note the risk of steroid-induced psychosis at these doses, so enrollment in rheumatological clinic/ward is recommended.

    The pulse infusion is given as a single dose with 30-60 minutes of infusion time. The prescribed amount of Solu-Medrol is added to 100 ml of sodium chloride 9 mg/ml. Pulse and blood pressure are checked before, after half the infusion is administered and after the completion of the infusion, and after another 1-2 hours.
  • Without visual symptoms
    Prednisolone po 30-40 mg/day in a single dose until 2 weeks after symptom release, after which the daily dose is gradually reduced at the same rate as described below under the heading “tapering”.

If a significant improvement, but not symptom relief is achieved with the initial dose, the patient will be asked if symptomatic relief is present from noon through the night. In this case, do not increase the daily dose, but redistribute to a 2-dose regimen with a higher dose in the morning, approximately distribution 60% + 0 + 40%.

Tapering of prednisolone

  1. Reduce the daily dose by 5 mg every other – every three weeks to 20 mg/day.
  2. Reduce daily dose by 2.5 mg every other – every 3 weeks to 10 mg / day.
  3. Reduce the daily dose by 1.25 mg every month to 0.
  4. Prepare the patient for clinical relapse – increase the dose one step above the last dose that gave symptom relief, and then follow the withdrawal schedule. In the case of visual symptoms pulse infusion as above.

Expect a treatment duration of 1½ – 2½ years before the successful completion of the therapy can occur.

  • During the entire treatment period the dose should be kept minimal and the patient symptom-free.
  • One-day dosing of prednisolone is not appropriate for TA.
  • Steroid therapy always involves the risk of agitation, insomnia, night sweats, “stretch marks” and in the long term, especially osteoporosis. Steroid therapy also leads to the induction of diabetes, the progression of cataracts, and osteonecrosis of the hip.
  • All patients should receive osteoporosis prophylaxis through smoking cessation, encouraging outdoor physical activity (sunlight). Prophylactic effects of calcium and vitamin D supplements have been questioned. Osteoporosis prophylaxis with bisphosphonate should be prescribed to everyone if there is no contraindication.
  • There is no lowest harmless steroid dose considering osteoporosis development and risk of vertebral compression.
  • Never hesitate to give very high doses of steroids in vision impairment. Admittedly, it is doubtful if vision loss is reversible, but one protects the other eye from ischemic damage.

In the event of difficulties with adequate prednisolone dosing

Tocilizumab (Roactemra) has been approved for use in giant cell arteritis in 2017, but so far, experience for use in clinical practice is limited. The therapy may be relevant in severe steroid complications, other conditions where long-term steroid therapy is inappropriate. Until now, treatment should only be prescribed in a unit with the habit of handling immunosuppressive biological drugs.

Follow-up – Temporal Arteritis

The patient’s symptoms determine the cortisone dose. It is therefore important that the same physician maintains continuous contact with the patient in order to be able to evaluate the nuances of the patient’s symptom progression. Strive for symptom relief and minimize the steroid dose gradually. At each dose change:

  • Medical History regarding symptom recurrence
  • Medical examination – bilateral radial pulses, blood pressure in both arms and auscultation of the carotid vessels
  • SR; B-glucose or P-glucose
  • There are recent studies that have shown a positive effect of tocilizumab (RoActemra) in temporal artery disease when it becomes difficult to reduce cortisone. For the time being, this therapy should be reserved for use in rheumatology clinics with experience administering this medication.

Other

  • If the patient regains typical symptoms and SR is normal, increase the steroid dose. Cortisone medication often causes SR <10 mm. Do not forget that the current age group has normal values ​​for SR up to 30-35 mm. Thus, allow SR to reach these values ​​if the patient is symptom free.
  • If SR rises above the reference level in a symptom-free patient, consider and investigate other unrelated causes of SR progression or reevaluate the diagnosis.
  • In case of substantial dose changes, “steroid-release pseudorheumatism” occurs, which gives PMR-identical symptoms without being indicative of recurrence. If the dose reduction has been significant, wait without any action for a week.
  • Steroids have no curative effect but keep the inflammation suppressed during spontaneous healing, which in untreated cases occurs after 1.5-3 years. The expected treatment time is thus in this range.
  • Question the therapy or diagnosis if steroids are required for more than 3 years. Refer these cases to a rheumatologist for assessment.
  • TA has a fluctuating natural course, which means that relapse with symptom onset is common if the steroid dose is kept at a low level. In this situation, SR is of little use, as SR is normal in up to 50% of cases of recurrence. Adjust the dose to one step above the last dose that gave symptom relief.
  • The most common causes of treatment failure are premature attempts to terminate therapy or rapid dose reduction.

Further Reading