Giant cell arteritis characterised by sore throat

Abstract

Giant cell arteritis is a devastating condition that can lead to permanent vision loss. Early diagnosis is vital to initiate corticosteroid treatment before irreversible visual symptoms develop. This case study examines an 81-year-old woman with a history of polymyalgia rheumatica who initially presented with sore throat and headache. She was ultimately diagnosed with giant cell arteritis. However, her diagnosis was delayed by multiple emergency and outpatient providers who failed to evaluate her for giant cell arteritis. Her story reflects the importance of assessing polymyalgia rheumatica patients for giant cell arteritis even when presenting with more unusual symptoms, such as sore throat.

Background

Giant cell arteritis is a vasculitis that predominantly affects women over the age of 50. This condition’s exact cause is unknown, but there are likely genetic and infectious factors that contribute to its pathogenesis.¹ Headache is the most common presenting symptom of giant cell arteritis, appearing in at least 80% of all confirmed cases.^{1 2} Of those patients presenting with headache, 43.3% also experience jaw claudication, and 5.9% develop dysphasia.² About 40% of confirmed giant cell arteritis patients also present with polymyalgia rheumatica, which is characterised by arthritic pain in the hips and shoulders.¹ Other common symptoms associated with giant cell arteritis include constitutional syndrome, fever, and scalp tenderness.²

The most feared complication of giant cell arteritis is irreversible vision loss, but giant cell arteritis can also produce other ischaemic manifestations, including stroke or limb claudication.² The best positive predictor of vascular complications for giant cell arteritis patients is an abnormal temporal artery on physical examination.² Tenderness to palpation of the external carotid has been very rarely seen with giant cell arteritis patients, but this finding may also be associated with vascular complications.³ However, anaemia may be protective against vascular complications when present at the time of diagnosis of giant cell arteritis.²

Giant cell arteritis is treated with steroid therapy, which usually resolves all symptoms apart from vision loss. Medium dose steroids are typically recommended over high dose steroids for giant cell arteritis, because they have fewer negative effects without any significant change in therapeutic benefit.⁴ Patients with higher cardiovascular risk or ischaemic complications may benefit from aspirin therapy for at least a month after diagnosis of giant cell arteritis.⁵ Preventative treatment for osteoporosis is also recommended to counteract the effects of steroid treatment for giant cell arteritis.¹

Case presentation

The patient was diagnosed with polymyalgia rheumatica 8 years ago. She has been taking a maintenance dose of prednisone 3 mg daily for years. She more recently awoke in the middle of the night with sudden onset of severe sore throat and bilateral temporal headache with radiation to the neck and jaw. She also had general fatigue, difficulty chewing and some sudden vision changes described as blurry vision. She presented to a local emergency department with these symptoms, where she had a negative COVID-19 test. A comprehensive metabolic panel (CMP) and a complete blood count (CBC) were unremarkable apart from mild normocytic anaemia with a haemoglobin of 111 g/L. She was sent to a neurologist, who ruled out stroke. An oral surgeon found no abnormalities. She then saw an ear, nose and throat (ENT) specialist, who diagnosed her with carotidynia and prescribed gabapentin 300 mg daily. She subsequently presented these symptoms to her rheumatologist, who suspected giant cell arteritis. She was found to have an elevated C reactive protein (CRP) of 6.1 mg/L, and an erythrocyte sedimentation rate (ESR) of 41 mm/hour. Her prednisone was increased to 40 mg daily, and her symptoms rapidly improved. Aspirin was considered, but ultimately decided against when coronary angiography revealed no significant stenoses.

Two weeks later, she presented to general surgery for bilateral temporal artery biopsies. She continued to have mild pain in her neck in spite of the higher prednisone dose. Her body mass index was 22.8 kg/m², blood pressure was 125/86 mm Hg and heart rate 77 bpm. Physical examination revealed a palpable left temporal pulse at the hairline ascending a few centimetres, and a barely palpable right temporal pulse at the hairline without ascension. Her right temporal scalp may have had a slightly non-tender firm area on palpation. Her neck was supple without jugular venous distention (JVD). Respiratory and cardiovascular examinations were unremarkable. Bilateral temporal artery biopsies were performed, demonstrating grossly abnormal temporal arteries with visible pale segments. Separating the arteries from their smaller branches produced minimal bleeding, indicating slow blood flow. Microscopic analysis of both arteries revealed segmental transmural inflammation, disruptions in the internal elastic membrane, giant cells and intimal hyperplasia. The biopsies were therefore both positive for giant cell arteritis.

Outcome and follow-up

She continued to report mild neck pain 2 weeks after the temporal artery biopsies. However, this pain was much improved compared with her initial presentation. She discussed discontinuation of gabapentin with her ENT physician after her diagnosis of giant cell arteritis was confirmed. She has since stopped this medication without recurrence of symptoms. Her sore throat has now resolved, and her only lasting symptom is slightly blurry vision that still bothers her when reading for a prolonged period of time. Her rheumatologist will continue to follow her medical regimen, and she will have continued ESR and CRP drawn as she tapers off steroids over the coming months.

Discussion

This case report demonstrates the importance of identifying giant cell arteritis from the relatively uncommon symptoms of sore throat and carotid tenderness, especially for patients with a history of polymyalgia rheumatica, in order to initiate treatment before irreversible visual symptoms develop.

Giant cell arteritis has a wide variety of common presenting symptoms, including but not limited to headache, jaw claudication and vision changes.¹ It can rarely present with respiratory symptoms, such as cough, hoarseness or sore throat. Respiratory involvement is suspected to stem from pathological changes to the ascending pharyngeal branches of the external carotid artery.⁶ Because her sore throat improved along with her more classic symptoms when she increased prednisone, her sore throat was likely also related to giant cell arteritis.

The carotid tenderness that prompted her initial diagnosis of carotidynia was also most likely a rare manifestation of giant cell arteritis, which can indicate more severe disease progression with higher likelihood for vascular complications.³ However, anaemia may have a protective benefit against developing vascular complications.² Therefore, this patient’s normocytic anaemia with a haemoglobin of 111 g/L may have contributed to her lack of vascular complications in spite of her severe disease progression with carotid tenderness.

Giant cell arteritis has also been linked to polymyalgia rheumatica either simultaneously or in sequence. There are many theories as to the basis of this connection, ranging from genetic associations with HLA-DRB1*04 alleles to various bacterial and viral infectious agents.¹ There is some controversy regarding whether these are in fact two separate conditions or different manifestations of the same pathology.¹ In either case, it is reasonable to assume that the patient’s prior polymyalgia rheumatica gave her an increased likelihood of developing giant cell arteritis at some point in her life.

Her maintenance dose of prednisone 3 mg daily for polymyalgia rheumatica would not likely be sufficient to prevent the development of giant cell arteritis. Giant cell arteritis patients who experience a recurrent episode are already taking an average corticosteroid dose of 7.8 mg daily.⁵ Another study showed no significant difference in relapse rates of giant cell arteritis between patients taking medium dose steroids and those taking high dose steroids.⁴ Therefore, there was no reason to discount development of giant cell arteritis in this patient taking a very small dose of daily prednisone.

This case establishes the importance of assessing polymyalgia rheumatica patients for giant cell arteritis even when presenting with atypical symptoms. Sore throat and carotid tenderness are rarely associated with giant cell arteritis, but they are associated with more severe vascular complications. This patient’s diagnosis was delayed when giant cell arteritis was not considered at her initial presentation, which could have been detrimental to her quality of life had she developed significant visual symptoms before receiving treatment.

Patient’s perspective.

The patient has provided the following statement regarding her experience with giant cell arteritis, lightly edited for clarity.

One evening I awoke from my sleep feeling like a bomb was going off in my head. In addition to that continued head pain, my throat was extremely sore, and my jaw was tight and painful. I went to an emergency walk-in clinic, but they were really unable to help me. The next day I saw my oral surgeon, thinking that it could be a problem emanating from my mouth. He found no issues. I then went to see an ENT who believed I had throat neuropathy. In the meantime, I was in constant pain, so I went to another emergency room, where I was suggested to see a neurologist. I made an appointment and this doctor said he believed that I did in fact have giant cell arteritis, but he confirmed that the doctor I needed to see was my rheumatologist.

Apparently, I was very lucky to have seen my rheumatologist so quickly, because if not rapidly addressed, blindness normally occurs. He put me on 40 mg of prednisone and made arrangements with a surgeon to biopsy both sides of my head at the arterial location. The result was positive that I have giant cell arteritis. I maintained the 40 mg of prednisone for a little over a month, then the dosage was reduced every few weeks, and I am presently taking 25 mg daily. Needless to say, I am grateful for the care I received and the outcome. This is a very painful and scary diagnosis, but thankfully I have completely recovered with no lingering symptoms.

Learning points.

• Early diagnosis and treatment of giant cell arteritis is crucial for avoiding permanent vision loss or other severe vascular complications.

• Carotid tenderness is a rare manifestation of giant cell arteritis, but this symptom is often associated with a more severe disease progression. Therefore, carotid tenderness should always prompt evaluation for giant cell arteritis.

• Polymyalgia rheumatica has a strong association with giant cell arteritis both simultaneously and in sequence. Evaluation for giant cell arteritis should be considered in polymyalgia rheumatica patients presenting with less common respiratory symptoms, such as sore throat.

Ethics statements

Patient consent for publication

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