Headache — Clinical Case

DCE Long Case

Patient brief (provided to trainee)

Patient: Mr Robert Chen, 74 years old, retired carpenter. [1]

Presenting complaint: Referred by his general practitioner with a 3-week history of new headache and 2 days of transient visual loss in the left eye. [1]

History of presenting complaint: Three weeks ago, Mr Chen developed a new left-sided temporal headache, constant and aching, worse in the mornings. He noticed pain in his jaw when eating his dinner, forcing him to stop after a few mouthfuls (jaw claudication). Two days ago, he had a 10-minute episode of complete greying-out of vision in his left eye while reading — transient visual obscuration. He has felt generally unwell, with fatigue, mild fever, and a 3 kg weight loss over the past month. His scalp is tender when he combs his hair. [1]

Past history:

• Polymyalgia rheumatica, diagnosed 8 months ago, currently on prednisolone 8 mg daily (tapering from an initial 15 mg)
• Ischaemic heart disease — prior non-ST elevation myocardial infarction 5 years ago; on aspirin, atorvastatin, bisoprolol
• Hypertension — on ramipril
• Type 2 diabetes — on metformin
• Osteoarthritis of the knees
• No known drug allergies [1]

Medications: Prednisolone 8 mg daily, aspirin 100 mg daily, atorvastatin 40 mg nocte, bisoprolol 5 mg daily, ramipril 5 mg daily, metformin 500 mg twice daily, calcium-vitamin D supplement, alendronate 70 mg weekly. [1]

Examination findings (trainee elicits):

• Alert and oriented, GCS 15. Appears mildly uncomfortable.
• Temperature 37.8 degrees, heart rate 88 (regular), blood pressure 145/82, respiratory rate 16, oxygen saturation 97 per cent on room air.
• Temporal arteries: The left temporal artery is thickened, tender, and has a diminished pulse. The right temporal artery is palpable and non-tender. No bruits audible.
• Fundoscopy: The left optic disc shows mild pallor with blurred superior margin (possible early anterior ischaemic optic neuropathy). The right disc is normal. No papilloedema. Visual acuity is 6/9 on the right and 6/18 on the left.
• Neurological examination: No focal motor or sensory deficit. Cranial nerves otherwise intact (no sixth nerve palsy). Coordination and gait normal.
• Musculoskeletal: Mild proximal muscle tenderness in the shoulder girdles consistent with active PMR. No true weakness.
• Cardiovascular, respiratory, abdominal examination: Normal. [1]

Investigations (available in the case):

• Full blood count: white cells 12.1 (neutrophils 9.4), haemoglobin 112 (normocytic), platelets 410.
• ESR 62 mm per hour, CRP 48 mg/L.
• Sodium 134, potassium 4.2, urea 8.1, creatinine 95, eGFR 62.
• HbA1c 58 mmol/mol (slightly raised from baseline of 54 — attributed to steroids).
• Glucose (fasting): 8.2 mmol/L.
• Liver function: mild elevation of alkaline phosphatase at 140 (consistent with GCA).
• CT head: age-appropriate atrophy, no mass, no haemorrhage. [1]

Candidate's structured presentation (model)

Opening statement: [1]

"Mr Chen is a 74-year-old retired carpenter who presents with a 3-week history of a new left temporal headache, jaw claudication, scalp tenderness, constitutional symptoms, and a 2-day history of a transient visual obscuration in the left eye, in the context of known polymyalgia rheumatica on tapering prednisolone. He has ischaemic heart disease, hypertension, and type 2 diabetes. [1]

His main problems are:

1. Giant cell arteritis — new temporal headache, jaw claudication, raised ESR and CRP, abnormal temporal artery, and threatened vision (transient visual obscuration with a pale left optic disc) — this is a rheumatological emergency requiring immediate high-dose steroids
2. Polymyalgia rheumatica relapse — the GCA has emerged as a flare of his underlying PMR during the prednisolone taper
3. Threatened blindness — transient visual obscuration and a pale left disc indicate evolving anterior ischaemic optic neuropathy; the risk of permanent visual loss is immediate
4. Ischaemic heart disease, hypertension, and type 2 diabetes — comorbidities that complicate high-dose steroid therapy (glycaemic control, fluid retention, cardiovascular risk)
5. Steroid side-effect management — he is already on bone protection and gastric protection; the steroid dose will need to increase significantly, with monitoring of diabetes and bone health [1]

My immediate plan is to admit Mr Chen, give intravenous methylprednisolone 500 mg daily for 3 days given the visual symptoms, then transition to oral prednisolone 60 mg daily, arrange temporal artery ultrasound and biopsy within 2 weeks, involve rheumatology and ophthalmology, and manage his comorbidities — particularly his diabetes, which will worsen on high-dose steroids." [1]

Management plan: [1]

1. Immediate high-dose steroid therapy (urgent): Intravenous methylprednisolone 500 mg daily for 3 days, then oral prednisolone 60 mg daily. The IV route is chosen because of the visual symptoms — anterior ischaemic optic neuropathy can progress to permanent blindness within hours, and IV steroids achieve higher tissue levels to protect the remaining vision and the contralateral eye. The oral prednisolone replaces his current 8 mg maintenance dose for PMR. Do not wait for temporal artery biopsy — the histology remains positive for at least 2 weeks after starting steroids. [1]

2. Temporal artery ultrasound and biopsy within 2 weeks: Arrange urgent temporal artery ultrasound (looking for the halo sign — a dark ring around the vessel lumen indicating wall oedema). If ultrasound is not available or inconclusive, arrange temporal artery biopsy within 2 weeks of starting steroids. Warn the patient that the biopsy can have skip lesions, so a negative result does not exclude the diagnosis. The 2022 ACR/EULAR criteria recognise both the halo sign on ultrasound and positive biopsy as the highest-scoring items (5 points each), and a cumulative score of 6 or above classifies the patient as having GCA. [1]

3. Ophthalmology referral (urgent): For formal visual acuity assessment, visual fields, optical coherence tomography, and baseline documentation. The ophthalmologist will monitor for progression of anterior ischaemic optic neuropathy and assess the contralateral eye. [1]

4. Rheumatology referral: For shared care of the GCA and PMR, guidance on the steroid taper (typically 12-18 months), and consideration of tocilizumab (IL-6 receptor antagonist) as a steroid-sparing agent, particularly given his diabetes, which will be difficult to manage on prolonged high-dose steroids. [1]

5. Diabetes management: Increase blood glucose monitoring to 4 times daily. Expect significant hyperglycaemia on 60 mg prednisolone. Involve the diabetes team. Likely need to uptitrate metformin and add a basal-bolus insulin regimen during the high-dose phase, with the plan to de-escalate as the steroid dose reduces. Consider an SGLT2 inhibitor for both glycaemic control and cardiovascular protection, if not contraindicated. [1]

6. Bone and gastric protection: Continue alendronate and calcium-vitamin D (he is already on these for PMR-related steroid therapy). Continue aspirin (no contraindication; may have a role in reducing visual loss in GCA, though evidence is limited). Add a PPI for gastric protection at the higher steroid dose. [1]

7. Steroid taper plan: After the initial 3-4 weeks at 40-60 mg, taper by 10 mg every 2 weeks to 20 mg, then by 1 mg every 2-4 weeks, guided by symptoms, ESR, and CRP. The taper will be prolonged (12-18 months) with regular monitoring. Relapses are common and present as a recurrence of headache or PMR symptoms with rising inflammatory markers. [1]

8. Communication and patient perspective: Explain to Mr Chen that his condition is a vasculitis of the temporal artery that threatens his sight, that the treatment is high-dose steroids which will have significant side effects (worsening diabetes, weight gain, mood changes, bone thinning), but that the alternative — blindness — is far worse. Involve his family in the discussion. Acknowledge that he is already familiar with steroids from his PMR and that the dose is increasing temporarily. Address his concerns about his diabetes and cardiovascular risk. [1]

Examiner discussion questions

Q: "His ESR is 62 and CRP is 48. Does this confirm giant cell arteritis, or could these be elevated from another cause?" [1]

"The markedly elevated ESR and CRP are strongly supportive of giant cell arteritis in this clinical context, but they are not diagnostic in isolation — they are non-specific markers of inflammation. In this patient, the combination of new temporal headache, jaw claudication, scalp tenderness, an abnormal temporal artery on examination, constitutional symptoms, raised inflammatory markers, and a visual symptom is a textbook presentation of GCA. The pre-existing PMR further supports the diagnosis — GCA and PMR are closely related conditions, and 15 to 20 per cent of patients with PMR develop GCA, often during the steroid taper. The liver enzyme abnormality (raised alkaline phosphatase) is also a recognised feature of GCA — it reflects the hepatic arterial involvement. The diagnosis will be confirmed or supported by the temporal artery ultrasound or biopsy, but the decision to treat is clinical and immediate." [1]

Q: "Why did the GCA emerge while he was on prednisolone 8 mg for PMR?" [1]

"This is a common and clinically important scenario. Prednisolone 8 mg daily is a low dose — sufficient to suppress the PMR symptoms but not sufficient to prevent the development of GCA. GCA can emerge during the PMR taper when the dose drops below the threshold needed to suppress the vasculitic component. The lesson is that any patient with PMR who develops a new headache, jaw claudication, or visual symptoms during the steroid taper must be assessed urgently for GCA, even if they are still on a low dose of prednisolone. The 8 mg was controlling the PMR but was inadequate to prevent the GCA flare. The management is to increase the prednisolone to GCA treatment doses (40-60 mg), which will also control the PMR." [1]

Q: "He has had a transient visual obscuration. What is the risk to his remaining vision, and how does it change your management?" [1]

"The risk is immediate and serious. A transient visual obscuration — a brief episode of visual loss in a patient with GCA — is a sentinel event. It indicates that the blood supply to the optic nerve has been compromised by the vasculitis, and anterior ischaemic optic neuropathy (AION) is imminent. Once AION occurs and the vision is lost, it does not recover. The risk to the contralateral eye is also significant — involvement of the second eye can occur within days. This is why I am using intravenous methylprednisolone 500 mg daily for 3 days rather than oral prednisolone — the goal is to achieve the highest possible steroid levels in the vessel wall as quickly as possible to halt the vasculitis and protect both eyes. The ophthalmology referral is to document the current visual status (baseline), to assess for AION (which may already be evolving given the pale left disc), and to monitor closely for progression." [1]

Q: "How would his diabetes management change on high-dose steroids?" [1]

"High-dose prednisolone causes significant insulin resistance and hepatic glucose output, typically producing postprandial hyperglycaemia with relatively preserved fasting glucose early on. My approach: increase his blood glucose monitoring to 4 times daily (pre-meals and bedtime), involve the diabetes team from day one, and anticipate the need for insulin. I would uptitrate his metformin to the maximum tolerated dose (2 g daily), and add a basal-bolus insulin regimen — a basal insulin (glargine or detemir) to control fasting glucose and rapid-acting insulin (lispro or aspart) with meals to control the postprandial spikes driven by the steroids. The insulin doses will need frequent review, particularly as the prednisolone is tapered — the requirement can drop rapidly as the dose falls. I would continue his metformin for its cardiovascular and insulin-sensitising benefits and consider an SGLT2 inhibitor if not contraindicated. His HbA1c is already elevated at 58 mmol/mol on his current 8 mg prednisolone, so I expect it will worsen on 60 mg. The goal is to prevent severe hyperglycaemia and ketosis, not to achieve perfect control — the steroids are life-sight-sparing and must be prioritised." [1]

Q: "What is the role of tocilizumab in this patient?" [1]

"Tocilizumab, an interleukin-6 receptor antagonist, is licensed for the treatment of giant cell arteritis. It reduces both the relapse rate and the cumulative steroid exposure, which is particularly valuable in this patient given his diabetes, which is already difficult to control on steroids. The GiACTA trial, published in the Lancet in 2017, showed that tocilizumab combined with a 26-week prednisone taper was superior to a 26-week or 52-week prednisone taper alone for sustained remission. In this patient, I would discuss tocilizumab with rheumatology after the initial steroid induction, particularly if his diabetes is proving difficult to manage or if he relapses on the steroid taper. The main considerations are the cost, the need for monthly subcutaneous injections, and the small risk of serious infection and bowel perforation. It would not replace the initial high-dose steroids — those are needed immediately to protect his vision — but it would allow a faster steroid taper and reduce the cumulative steroid burden." [1]

Q: "How would you counsel Mr Chen about his long-term outlook?" [1]

"I would explain that giant cell arteritis is a treatable condition, and that with prompt and adequate steroid therapy, his vision can be preserved and his headache and jaw symptoms will resolve within days. The challenge is the prolonged steroid course — typically 12 to 18 months — with its side effects, particularly the impact on his diabetes, his cardiovascular risk, and his bone health. I would reassure him that we have strategies to manage each of these: intensive diabetes management with the diabetes team, continuation of his cardiovascular medications, bone protection with alendronate, and the possibility of tocilizumab to reduce the steroid burden. I would warn him that relapses can occur, particularly during the taper, and that he should report any recurrence of headache, jaw pain, or visual symptoms immediately. I would also address his psychological wellbeing — a new diagnosis of a sight-threatening condition on top of existing comorbidity is stressful, and I would offer support and follow-up. Finally, I would discuss advance care planning in the context of his overall health, ensuring that his preferences for future care are documented." [1]