Temporal arteritis

Question 1

Temporal arteritis (TA) other names

Answer 1

a subset of giant cell arteritis (GCA)

Cranial arteritis.

Question 2

Presenting symptoms

Answer 2

Can vary and can be non-specific

Headache is the most common, 75 to 90% of cases.

• persistent unilateral throbbing headache
• often severe, in the temporal or occipital area,
• scalp sensitivity
• can be worse at night
• or presents as a new headache.

Usually >50 yrs; the mean age is 70 yrs.

Less common symptoms are:

1. Systemic features, e.g., low-grade fever, anorexia, and fatigue (50% of pts)
2. Scalp tenderness, especially when combing hair or resting on a pillow (25%).
3. Jaw claudication, esp when eating or talking, ( 25%).
4. Visual disturbances, such as blurred vision, diplopia, and amaurosis fugax, which are of critical importance.

Question 3

Red flag

Answer 3

Aged > 50 years with new-onset or a new type of headache

Question 4

Background

Answer 4

• An immune-mediated, ischaemic condition that is caused by inflammation in the wall of medium to large arteries.
• TA is usually the only artery in which physical changes are clinically apparent. Hence, the alternative name of temporal arteritis.
• Large-vessel stenosis occurs in 10 to 15% of pts.
• Usually >50 yrs; the mean age is 70 yrs.
• Most prevalent in Caucasians
• 2 to 3 times more common in females than males.
• 40 to 60% of pts have concurrent PMR
• 16 to 21% of people with PMR will develop TA.
• The mortality rate is not significantly different from the general population.
• However, the risk of aortic aneurysm is 17 times greater, even after timely and successful treatment.

Question 5

Examination

Answer 5

1. Localized thickening
2. hardened, nodular
3. with or without loss of pulsation of TA

A normal temporal artery does not exclude temporal arteritis.

Eye examination

Question 6

Eye examination

Answer 6

A). Visual acuity

1. Ask if pt has distance glasses with them, and if either eye has had known poor vision e.g., a lazy eye.
2. Test their distance vision – check each eye separately and together, with distance glasses if worn, using a chart at the correct distance.
3. If acuity is still subnormal, check with a pinhole.
4. If vision improves with a pinhole, and no cataract is present, recommend the pt arrange a review of their glasses.
5. If unable to read any letters on chart, assess the following in order:

• Finger counting
• Hand movements
• Light perception

B). Visual fields to confrontation (count fingers in four quadrants for each eye)

C). Pupillary light reaction

D). Fundoscopy (optic disc and retinal vasculature)

Question 7

Diagnosis

Answer 7

Biopsy and histological examination of the superficial TA.

Either ESR / CRP or both can be raised (ESR>RCP).

• Because of the significant potential for morbidity, request both in the initial presentation.
• Any elevation of CRP /ESR suggests TA in a pt with s/s.
• A small number of pts will have levels within normal ranges on at least one of the tests.
• If both CRP and ESR are normal, the likelihood of TA is reduced, but cannot be ruled out.

Other findings are non-specific e.g., leucocytosis, raised PLTs.

Question 8

Temporal artery biopsy referral

Answer 8

If new visual symptoms, call the Ophthalmology GP line on (09) 630‑9964 for urgent review.

If no new visual symptoms, follow the appropriate DHB process

• Contact the on-call rheumatology registrar via the switchboard, (09) 307‑2800.
• Biopsy will normally be performed within 10 to 14 days.
• Request non-acute rheumatology assessment to aid follow-up. State in your referral that you have discussed the case with the rheumatology registrar.

Question 9

Other diagnoses

Answer 9

PMR

Migraine

Intracranial haemorrhage

Herpes zoster

Transient ischaemic attack (TIA)

Other causes of acute vision loss

Temporomandibular joint disorder

Question 10

Treatment

Answer 10

A). Begin steroid therapy immediately if GCA is suspected, and arrange TA biopsy to confirm diagnosis.

• IV methylprednisolone may be given to pts with recent onset vision loss by referring urgently to Ophthalmology.
• Must be treated urgently due to the significant risk of permanent vision loss.
• Corticosteroids decreases the risk of vision loss from 20% to 1%.
• Risk of blindness is highest for the first 3 months and then becomes low risk.

1. Advise about risks with steroids:
   
   • Arrange osteoporosis prophylaxis.
     
     • Request bone density scan, and consider starting bisphosphonates.
     • In an older, frail person, where osteoporosis is likely and access to bone densitometry delayed, consider starting a bisphosphonate (risedronate) while awaiting densitometry, as bone loss is said to occur in the first 3 months.¹
     • Advise about adequate dietary calcium intake and consider vitamin D replacement, if relevant.
   • Warn about side-effects.
   • Give stress or sick day advice/managementof long-term steroids.
2. In pts who are at high risk of steroid complications or who are resistant to steroid treatment, request rheumatology assessment for consideration of steroid‑sparing therapy e.g., low dose oral methotrexate.

B) Start low-dose Aspirin (100 mg daily).

• unless there are specific contraindications.
• Aspirin has been shown to reduce cranial ischaemic complications of GCA, such as TIA or stroke.

C). Arrange follow-up.

D). Manage any relapse.

Question 11

Steroid therapy

Answer 11

• Initial medication is prednisolone 40–60 mg (o) daily
• initially for 2–4 wks
• then gradual reduction (max. 10%) wk intervals.
• Dose reduction and progress is monitored by the clinical state and ESR levels.
• Usually symptoms improve within a week.
• If a response is not observed, the dose may need to be increased once diagnosis is confirmed.
• Monitor by clinical response, usually by loss of headache rather than by CRP.
• The dose of prednisone may be reduced after 4 weeks, as follows.
  
  1. Reduce by 10 mg every 2 weeks, down to 20 mg, then
  2. Reduce by 2.5 mg every 2 to 4 weeks, to 10 mg, then
  3. Reduce by 1 mg every 4 to 8 weeks, provided there are no relapses.
• Even with slow prednisone reduction, 50% of pts will have relapses during the first 12 to 18 months.
• An isolated elevation of ESR/CPR (in the absence of clinical symptoms) is not a valid reason to increase the dose of prednisone.
• Most pts will require prednisone for 2 to 3 years.

Question 12

Steroids Side Effects

Answer 12

1. Insomnia
2. Weight gain
3. Skin thinning, easy bruising
4. delayed wound healing
5. Osteoporosis
6. Gastrointestinal ulceration
7. Hypertension
8. Muscle weakness, immunosuppression, diabetes (either new onset or reduced control)
9. Psychiatric disturbances:

• mania
• delirium
• depression (especially if pre‑existing mood disturbances or excessive alcohol intake) which are uncommon unless prednisone doses > 40 mg.

Question 13

Follow up

Answer 13

• Schedule first follow up within a few days of the initial consultation.
• Further follow ups are suggested at 1, 3, and 6/52 later, then every 3/12 for the duration of steroid Rx.
• Check there are no s/s of relapse, and monitor the adverse effects of steroid Rx.
• Monitor CRP or ESR (whichever was raised at initial assessment) monthly until steroid dose reduction commences.
• If both ESR and CRP were raised at presentation, monitor CRP.
• Ask the pt to return if symptoms of TA, or steroid-related adverse effects occur between visits.
• There is a small risk of developing thoracic and abdominal aneurysms.
• A CXR at 1 year and 3 years post diagnosis could be considered.

Question 14

Manage relapse

Answer 14

Manage relapse by increasing the dose of prednisone.

• If TA suggestive headache, treat with the previous dose of prednisone. E.g, if the dose has been lowered to 10 mg daily and headache occurs, then revert dose back to 12.5 mg daily.
• If jaw claudication, return to 40 to 60 mg prednisone daily and consider further rheumatology assessment or advice.

If visual symptoms, treat with 60 mg prednisone and arrange prompt non-acute ophthalmology assessment.

Question 15

Long Term Glucocorticoids (Steroids)

Answer 15

A) Practice point

• Consider adrenal suppression in all patients on glucocorticoids in doses of > 5 mg prednisone per day for > 3 weeks.
• Warn patients about acute illness and the need to increase the steroid dose for 2 to 3 days.

B). Education:

• Discuss the risks with acute illnesses, trauma, or surgery due to HPA axis suppression and a lack of the normal cortisol response.
• Extra “stress or sick day dose steroids” may be needed.
• Give written advice.
• Diagnosis of serious or atypical infections may be delayed due to immunosuppression. Encourage early presentation if unwell.
• Consider MedicAlert.

C). Consider adrenal insufficiency

• e.g., nausea, fatigue, occasional vomiting, light-headedness when:
• the dose is reduced below the steroid equivalent of prednisone 5 mg per day.
• the patient is unwell, has trauma, or has undergone surgery.

D). Stress or “sick day” steroids

• i.e., situations when extra steroid doses are needed:
• Mild acute illness or trauma
• Moderate or severe acute illness (e.g., pneumonia) or significant vomiting or diarrhoea and reduced oral absorption, give parenteral hydrocortisone 50 to100 mg IV or IM and repeat 6 to 8 hourly. This may require hospital admission.

E). Withdrawal of steroids:

• Gradually reduce dose down to physiological dose of prednisone 5 mg or equivalent. The dose reduction will vary, and depends on the initial diagnosis.
• Once at prednisone 5 mg or equivalent, then either very slowly wean the steroid dose down, or follow these recommended steps and monitor recovery of normal steroid secretion.
• Warn the pt not to stop steroids suddenly and to seek help if they become unwell as the steroid dose will need to be increased.
• If there is difficulty weaning the pt off the steroids then follow these recommended steps and monitor recovery of normal steroid secretion.
• If pt is unable to stop steroids, request non-acute endocrinology assessment or seek endocrinology advice.