Endgames Case Review

Sequential vision loss in a patient with headache

BMJ 2016; 353 doi: http://dx.doi.org/10.1136/bmj.i3055 (Published 08 June 2016) Cite this as: BMJ 2016;353:i3055
  1. Siegfried Wagner, specialist trainee in ophthalmology1,
  2. Saurabh Jain, consultant ophthalmologist1
  1. 1Department of Ophthalmology, Royal Free Hospital NHS Foundation Trust, London NW3 2QG, UK
  1. Correspondence to: S Wagner siegfried.wagner{at}ndcn.ox.ac.uk

A 74 year old woman with a history of bowel cancer and hypertension was admitted under the general medical team from the emergency department because of a constant frontal headache for several weeks and poor oral intake secondary to jaw pain, which was exacerbated by chewing. She described intermittent blurring of vision in her left eye, which had culminated in complete loss of vision three days before presentation. She felt generally unwell with increasing lethargy and generalised myalgia.

On clinical examination she had bilateral tender and pulseless temporal arteries. Fundoscopy of the left eye showed features consistent with central retinal artery occlusion.

Initial blood tests showed raised inflammatory markers (C reactive protein (CRP) 2438 nmol/L, erythrocyte sedimentation rate (ESR) 116 mm in the first hour), and thrombocytosis (platelets 588×109/L). Despite symptomatic and biochemical improvement after prompt institution of appropriate treatment, her right eye deteriorated three days later exhibiting the features seen in the retinal photograph (fig 1). The optic nerve had a swollen pale “chalky white” appearance suggestive of an anterior ischaemic optic neuropathy.

Figure1

Fig 1 Retinal photograph

Questions

  1. What is the diagnosis?

  2. How would you confirm or investigate this diagnosis?

  3. What red flag symptoms should general practitioners be aware of?

  4. How would you treat this condition?

Answers

1. What is the diagnosis?

Short answer

Giant cell arteritis.

Discussion

The combination of the patient’s symptoms; the presence of thrombocytosis, raised CRP, and raised ESR; and the fundoscopic appearances is highly suggestive of giant cell arteritis (GCA). Patients with GCA are on average 70 years of age, with a female sex bias, and many have pre-existing polymyalgia rheumatica.1 Symptoms include headache, which can be generalised or localised to the temple, frontal, or occipital region; jaw claudication; and scalp tenderness. Vague constitutional symptoms of malaise, fever, and weight loss may also be present. The temporal arteries may be thickened, tender, and pulseless on palpation. GCA can also cause orbitopathy with ophthalmoplegia. Oculomotor palsies such as scalp gangrene can occasionally be the presenting feature.2

Arteritic anterior ischaemic optic neuropathy, as seen in this case, occurs in 20-50% of patients with untreated GCA. The second eye is also affected in 30% of patients, often within the first week.3 4

2. How would you confirm or investigate this diagnosis?

Short answer

Temporal artery biopsy, which should be performed promptly, is the diagnostic gold standard. It shows an inflammatory infiltrate and the presence of giant cells.

Discussion

In the past, temporal artery biopsy has been essential in confirming the diagnosis of temporal arteritis. Samples should ideally be greater than 2 cm and biopsy should be performed within seven days of starting steroids, although the yield may improve with earlier biopsy. Temporal arteries in patients with GCA often exhibit skip lesions. Although the procedure is usually carried out on the same laterality as the affected eye, it has been suggested that a contralateral biopsy should also be performed in patients with a negative result but a high degree of suspicion.5

In 75-90% of cases, colour Doppler and duplex ultrasonography show features consistent with temporal arteritis.6 7 Necrotising arteritis creates oedema within the artery wall, which manifests as a hypoechoic “halo” sign. The procedure has the advantage of being quick and readily available, although its sensitivity depends on the quality of the images and experience of the operator. Ultrasound features are present for an average of two weeks after steroids are started, and some centres use the degree of oedema as a surrogate for GCA activity.8

More recently, positron emission tomography (PET) has been used to characterise extracranial involvement in patients with GCA. The emission of paired gamma rays by a tracer, usually fluorodeoxyglucose (FDG), is used as a measure of disease activity, with a resolution of >4 mm.9 Thus, uptake by temporal vessels may be insufficient, but increased uptake by other large vessels, such as the aorta and subclavian arteries, may guide diagnosis. Moreover, PET can help detect complications of GCA and determine disease activity and response to treatment.10

3. What red flag symptoms should GPs be aware of?

Short answer

Suspect GCA in patients aged 50 years or more, especially those with polymyalgia rheumatica, new onset headache, or abnormality of the temporal artery (thickening, lack of pulsation, or tenderness). Other symptoms include visual disturbance, scalp tenderness, jaw claudication, and systemic features such as low grade fever, weight loss, and malaise.

Discussion

The National Institute for Health and Care Excellence recommends that GCA should be suspected in any patient aged 50 years or more (although it is uncommon in patients under 60 years) with a new onset headache, whether diffuse or localised to the temple.11 Abnormalities of the temporal artery, such as thickening, lack of or reduced pulsation, or tenderness should be explored. Other features suggestive of GCA include visual disturbance, scalp tenderness, and jaw claudication. Many patients show generalised systemic upset with weight loss, depression, anorexia, malaise, and lethargy. Less commonly, patients have neurological features such as cranial nerve palsies and transient ischaemic attacks. Patients often have pre-existing polymyalgia rheumatica so should be asked about a history of proximal muscle ache and morning stiffness. Primary care physicians should consider obtaining an urgent opinion from (or referral to) rheumatology services, with additional input from ophthalmology if the patient has visual symptoms. Blood tests—to look for raised CRP, ESR, and platelets—can facilitate the diagnosis. Patients with a high suspicion of GCA can be started on oral prednisolone in the community, although subsequent urgent referral to rheumatology is advised.

The American College of Rheumatology criteria can be used to confirm the diagnosis of GCA. The criteria include:

  • Age ≥50 years at time of onset

  • New onset headache

  • Temporal artery tenderness to palpation or reduced pulsation

  • ESR >50 mm in the first hour

  • Abnormal temporal artery biopsy.

The presence of three or more criteria has 93% sensitivity and 91% specificity for the diagnosis of GCA.12

4. How would you treat this condition?

Short answer

Steroids are the mainstay of treatment—0.5-1 g/day intravenous methylprednisolone in patients with visual symptoms, or 40-60 mg/day oral prednisolone if visual symptoms are lacking, together with bone and gastric protection agents. Treatment should ideally start immediately in patients with visual deterioration and within 24 hours in others.

Discussion

The cornerstone of treatment in GCA is prompt systemic corticosteroid therapy, either intravenous methylprednisolone (1 g/day) or oral prednisolone (40-60 mg/day).13 Intravenous methylprednisolone should be used in patients with ocular involvement, with oral therapy reserved for mild disease with no visual symptoms. Oral prednisolone can be started in the community, with subsequent management and dose tailoring by rheumatology and ophthalmology services.

Patients presenting with central retinal artery occlusion or arteritic anterior ischaemic optic neuropathy secondary to GCA can be started on three days of intravenous steroids, with subsequent conversion to a 1 mg/kg/day oral regimen. Steroids should be tapered gradually, titrated to clinical symptoms and biochemical parameters, ideally in conjunction with rheumatology and ophthalmology services. The British Society for Rheumatology has produced a useful tapering algorithm.14 Any relapse should be immediately be treated by a swift increase in corticosteroids. Patients should be counselled on the adverse effects of steroids, both short term and long term, and also be prescribed appropriate gastric protection, such as a proton pump inhibitor, and bone protection agents (eg calcium, vitamin D supplements, and bisphosphonates). Aspirin or alternative antiplatelet agents should be considered as they reduce the risk of ischaemic events in patients with GCA, although it is unclear whether this results from a direct effect on the disease process or the high rates of peripheral vascular and ischaemic heart disease in patients with GCA.15 The responsible clinician should also consider the impact of high dose steroids on patients with a background of diabetes or mental health disease. If symptoms persist despite confirmed GCA and subsequent high dose corticosteroids, further immunosuppression may be considered by the rheumatology department; however, this scenario is unusual.

As in other conditions that warrant long term corticosteroid treatment, steroid sparing agents should be considered after the acute phase of GCA has subsided in patients with recurrent relapses or when glucocorticoids have adverse effects. The evidence base is largest for methotrexate, but azathioprine, cyclophosphamide, and more recently tocilizumab, an anti-interleukin 6 monoclonal antibody, can also be considered.16 17

Patient outcome

The patient was treated with 1 g/day intravenous methylprednisolone for four days and then 60 mg/ day oral prednisolone thereafter. Temporal artery biopsy showed the presence of an inflammatory infiltrate and giant cells, and computed tomography-PET scanning showed increased uptake of FDG within the temporal arteries, internal carotid vessels, and ascending aorta. Although her symptoms and blood test results improved with treatment (platelets 290×109/L, C reactive protein 114 nmol/L, ESR 18 mm in the first hour), the changes in her vision were irreversible and her vision remained poor.

Footnotes

  • Competing interests: We have read and understood BMJ policy on declaration of interests and declare the following interests: None.

  • Provenance and peer review: Not commissioned; externally peer reviewed.

  • Patient consent obtained.

References

View Abstract