A Headache with Consequences - Temporal Arteritis

This will mark the third and final recap of the March 2019 asynchronous small group cases.  Feel free to take a look and listen back at Parts 1 and 2.  This post we’ll be covering the presentation fo a middle aged female with a headache.  The differential is broad and fraught with a number of life/limb/sight threatening diagnoses.  As with the previous recaps, we recorded a podcast where we walk through the presentation, initial work up and management.

The case

A 54 yo female with PMHx of HTN presents to your department with a headache.  Three days ago she began having this headache which has been primarily located on the right side of her forehead.  She says it’s constant and not alleviated by Ibuprofen or other home remedies. She describes a sense of dizziness that accompanies it and that she has had a recent dry cough (though otherwise feels fine).  She tells you she is mostly worried because her neighbor was diagnosed with carbon monoxide poisoning last year and she remembers her having a headache at that time.

  • Vitals 99 F, HR 87, 145/89, RR 13, SpO2 99%

When is a headache (not) just a headache?

The Emergency Medicine provider is tasked with quickly sorting out those conditions with the greatest morbidity and mortality.  Headache is a common presentation to the Emergency Department where the specter of subarachnoid hemorrhage, intracranial hemorrhage, meningitis, dural venous thrombosis, intracranial mass and other serious conditions loom large.  The focused history for patients presenting with a headache should include an inquiry into the following:

  • Timing - What is the time course of this headache?  Is it acute (within the past several hours)? Subacute (over the course of the past several days/weeks)? Chronic (ongoing for years, often recurring)?  

  • Previous headaches - Does the patient have a known history of headaches? Do they have a diagnosed history of migraines?  What was the quality, characteristics, and timing of previous headaches? In what way is this headache similar to or dissimilar from previous headaches?

  • Associated symptoms - Presence of true vertigo, fever, visual changes/loss of vision, alteration of mental status, focal neurologic findings, or seizures can all be highly concerning for a more serious cause of headache.

Table 1 Common Causes of Headache   Recreated from Table 32-1 Davis, V. Headache. Emergency Medicine: An Approach to Clinical Problem Solving, 2nd Edition. Chapter 32. pp 539.

Table 1 Common Causes of Headache

Recreated from Table 32-1 Davis, V. Headache. Emergency Medicine: An Approach to Clinical Problem Solving, 2nd Edition. Chapter 32. pp 539.

In general, providers should be especially alert for headaches that have an abrupt onset (maximal intensity within seconds to minutes), that differ in quality or character from the patient’s previous headaches, headaches occurring in patient’s who do not typically have headaches, and headaches with significant associated symptoms.  A thorough history and complete neurologic exam is mandated for all patients presenting to the ED with a headache.

Let’s Hear More From Our Patient

As we heard already, the headache was not abrupt in its onset.  She has noticed it off and on over the past several days.  She denies any preceding trauma.  She has had headaches in the past but this is more unilateral and persistent than her previous headaches.  Denies a change in vision, photophobia, sonophobia.  She states she doesn’t want to eat because it makes her headache worse.  Denies fever.

On physical exam, you note some tenderness along the right temple and apart from that, the patient has a completely normal physical exam and complete neurologic exam.

Let’s Distill it Down

You have a 54 yo F presenting to your ED with complaint of subacute onset of headache that is unilateral, right temporal in location, differs from any previous headaches, is worsened by eating.  On exam she has temporal tenderness but an otherwise normal exam.

Based on this history and physical you are concerned about temporal arteritis.  So how do you finalize your diagnosis? Is there any other testing you need to perform? Do you need to start any therapies? When should you involve a consultant? Does the patient require admission or specific outpatient follow up?  And what is temporal arteritis anyhow…

Temporal Arteritis

Temporal Arteritis (or Giant Cell Arteritis) is a chronic, granulomatous vasculitis of medium and large arteries, most commonly affecting the aorta, branches of the ophthalmic artery, and extra-cranial branches of the carotid arteries. (2) The diagnostic criteria for Giant Cell Arteritis (GCA) as defined by the American College of Rheumatology are seen in Table 2.  Presence of 3 or more factors has a sensitivity of 93.5% and specificity of 91.2% for the diagnosis of Giant Cell Arteritis. (2) Notably, a temporal artery biopsy is NOT needed for the diagnosis of Giant Cell Arteritis to be made.  

Table 2 ACR Diagnostic Criteria for Giant Cell Arteritis

Table 2 ACR Diagnostic Criteria for Giant Cell Arteritis

The incidence of GCA increases with increasing age, with most cases occurring in patients >50 yo and with a peak incidence at 80 years of age. (2)  It is more common in women as opposed to men and is more common in those of Nordic, Scandinavian, or Northern-European descent.  GCA causes ocular and systemic symptoms with 50% of patients having ocular complaints (amaurosis fugax, diplopia, pain) at the time of diagnosis. (2) Jaw claudication secondary to masseter ischemia is the symptom most predictive of a positive temporal artery biopsy.

GCA can affect both medium and large arteries.  While we tend to think of symptoms related to the medium vessel involvement, large vessel involvement is present in 80% of cases at time of autopsy. (3). Large vessel GCA can lead to arm claudication and pulse discrepancies (through involvement of the subclavian artery).  Involvement of the aorta may not lead to symptoms outside of the constitutional systemic symptoms present in polymyalgia rheumatica.  CT angiography or MRA can be used to radiographically identify large vessel involvement. (3,4)

Systemic corticosteroids are the mainstay of treatment for GCA.  Maintaining a high clinical suspicion, early diagnosis, and early treatment are key to treatment and hopefully preventing permanent vision loss. (2)


References

  1. Davis, V. Headache. Emergency Medicine: An Approach to Clinical Problem Solving, 2nd Edition. Chapter 32. pp 539.

  2. Baig IF, Pascoe A, Kini A, Lee AG. Giant cell arteritis: early diagnosis is key. Eye and Brain 2019;Volume 11:1–12. 

  3. Koster MJ, Matteson EL, Warrington KJ. Large-vessel giant cell arteritis: diagnosis, monitoring and management. Rheumatology 2018;57(suppl_2):ii32–ii42. 

  4. Keser G, Aksu K. Diagnosis and differential diagnosis of large-vessel vasculitides. Rheumatology International 2019;39(2):169–85. 


Authorship

Written by Jeffery Hill, MD MEd