|IMAGES IN RHEUMATOLOGY
|Year : 2017 | Volume
| Issue : 3 | Page : 177-178
Temporal arteritis with a normal erythrocyte sedimentation rate
KM Mohammad Iqbal1, Faeez Mohamad Ali2, Arun Oommen3, Jayasree Govindhan4, Chippy Eldhose1, Muhammed Jasim Abdul Jalal1
1 Department of Internal Medicine and Rheumatology, VPS Lakeshore, Kochi, India
2 Department of Cardiology, Government Medical College, Trivandrum, Kerala, India
3 Department of Neurosurgery, VPS Lakeshore, Kochi, India
4 Department of Pathology, VPS Lakeshore, Kochi, India
|Date of Web Publication||08-May-2017|
Muhammed Jasim Abdul Jalal
Department of Internal Medicine and Rheumatology, VPS Lakeshore Hospital, NH 47 Bypass, Maradu, Nettoor PO, Kochi - 682 040, Kerala
Source of Support: None, Conflict of Interest: None
Keywords: Disrupted internal elastic lamina, Giant cell arteritis, Headache, Temporal headache
|How to cite this article:|
Mohammad Iqbal K M, Ali FM, Oommen A, Govindhan J, Eldhose C, Jalal MJ. Temporal arteritis with a normal erythrocyte sedimentation rate. Indian J Rheumatol 2017;12:177-8
A 72-year-old female presented with sudden onset severe right-sided dull and throbbing temporal headache of 2-week duration. She had associated progressive blurring of vision, which eventually led to partial right eye blindness.
The patient was hemodynamically stable. She had right temporal artery tenderness. Visual acuity in the right eye was 5/60 whereas it was normal in left eye. Fundus examination was normal apart from the pale optic disc. Peripheral pulsations and blood pressure were symmetric in all extremities.
Erythrocyte sedimentation rate (ESR) was 20 mm/h. Chest radiograph and urinalysis were normal. The patient was immediately started on 60 mg of prednisolone suspecting temporal arteritis clinically.
She had complete recovery of headache and partial recovery of visual loss within 24 h of initiation of steroid therapy. She underwent a right temporal artery biopsy within a week after the initiation of steroid therapy. Intraoperatively, the right temporal artery was thickened. Biopsy was consistent with temporal arteritis. Histopathology showed layer between tunica media and adventitia of a muscular artery [Figure 1] with chronic inflammatory cell infiltration. Disrupted internal elastic lamina was seen on the elastic Van Gieson stain [Figure 2].
|Figure 1: High power view showing layer between tunica media and adventitia of a muscular artery with chronic inflammatory cell infiltration, including multinucleated histiocytic giant cells. Dystrophic calcification is also seen. Background shows fibrosis (×40)|
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|Figure 2: The elastic Van Gieson stain highlights the disrupted internal elastic lamina (high power view, ×40)|
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Her symptoms are now under control with low-dose (1 mg) prednisolone for long-term maintenance treatment along with calcium and Vitamin D supplements for bone protection and a proton pump inhibitor for gastric protection.
Giant cell arteritis (GCA) has a prevalence of <1% in the general population. GCA is a large-vessel vasculitis. It usually affects individuals >50 years old. American College of Rheumatology criteria for diagnosis of GCA include
- Age >50 years
- New onset or new type of localized headache
- Elevated ESR >50 mm/h (by Westergren method)
- Tender temporal artery or decreased temporal artery pulsation, unrelated to arteriosclerosis of cervical arteries
- Biopsy specimen showing vasculitis characterized by mononuclear infiltration or granulomatous inflammation, usually with multinucleated giant cells.
The gold standard test to confirm the diagnosis is temporal artery biopsy. Initiation of medical treatment should be abrupt, and waiting for biopsy result should not delay the initiation of high-dose steroid treatment in a suspected case of GCA. The positivity of the biopsy results is dependent on multiple factors such as skip lesions and very small temporal artery sample.
Historically, ESR has been considered one of the most important markers to predict GCA. A normal ESR makes GCA unlikely, however does not rule it out. A meta-analysis of 114 studies showed that a high level of ESR was a less important indicator in ruling out GCA as the underlying cause for the patient's symptoms, but positive physical findings, characteristic of GCA, were more likely to be a strong indicator of a positive diagnosis of GCA. Our case had positive clinical findings such as temporal artery tenderness with a normal ESR which caused confusion among primary care physicians regarding the possibility of temporal arteritis.
Treatment includes immediate initiation of high-dose glucocorticoids. Visual loss is an early complication of the disease. Once established, it rarely improves. This emphasizes the need for early treatment in GCA. Prednisolone 40–60 mg daily is usually the recommended dose for 4 weeks. The total duration of high-dose prednisolone therapy depends on the resolution of symptoms., The dose is initially reduced by 10 mg every 2 weeks up to 20 mg, then by 2.5 mg every 2 weeks up to 10 mg, and then by 1 mg every 4–8 weeks, provided there are no further relapses.
If the patient presents with headache relapse, they should be restarted on the previous higher prednisolone dosage. Relapses with visual symptoms should be treated with either 60 mg prednisolone or intravenous methylprednisolone. Further investigations such as positron emission tomography and magnetic resonance imaging are recommended in large-vessel GCA relapses., Systemic vasculitis protocols should be considered in large-vessel GCA.
The diagnosis of temporal arteritis requires a high index of clinical suspicion. Atypical GCA presentations such as low ESR, arm claudication, and dysarthria should not be a barrier in suspecting GCA. Early and prompt initiation of high-dose steroid therapy in patients with temporal arteritis can prevent disastrous complications such as blindness, which once sets in is almost impossible to cure.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]