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Rapidly developing intimal fibrosis mimicking giant cell arteritis
  1. A Petzold1,
  2. G T Plant1,
  3. F Scaravilli2
  1. 1Moorfields Eye Hospital, City Road, London EC1 2PD, UK
  2. 2Department of Neuropathology, Institute of Neurology, Queen's Square, London WC1N 3BG, UK
  1. Correspondence to: Dr Petzold

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Temporal headache associated with a tender superficial temporal artery and decreased pulse on palpation are characteristics of giant cell arteritis. We report the clinical and biopsy findings in a patient in whom these symptoms were caused by a rapid developing intimal fibrosis.

Case report

A 51 year old woman presented with a 2 month history of a tender and painful left superficial temporal artery (STA). First she noticed a “thickened cord” on the left temple which felt pulsatile on palpation. Within 6 weeks the throbbing pulse disappeared and was followed by tenderness and pain. She also reported arthritic pain in the limbs and a morning cough over a 2 year period. She has smoked 40 cigarettes per day for 30 years. On examination the left STA felt hardened, knotted, non-pulsatile, and was slightly tender over a 2 cm distance (Fig 1, cross). There were no bruits on auscultation of major arteries. The facial and maxillary arteries were soft and pulsatile on both sides. The C reactive protein (CRP) was normal and the erythrocyte sedimentation rate (ESR) was 4 mm in the first hour. Blood pressure was 135/75.

Figure 1

Thickened, tender, and painful superficial temporal artery in a 51 year old woman. At the site of biopsy the artery was pulselessness (cross). A knotted appearance is also present distally and rostrally (arrow).

It was considered necessary to exclude an inflammatory cause, given the similarity of the presentation to giant cell arteritis. The biopsy was taken at the site where the STA was pulseless and most prominent (Fig 1). The tissue was examined using the serial block, serial section technique in order to minimise the chance of a false negative result. The histological appearances were those of a severely fibrosed artery devoid of any inflammatory changes and consistent with arteriosclerosis. The intima was considerably thickened, the internal elastic lamina was preserved, and the media had some degree of fibrosis (Fig 2). The thickened intima, which had led to tightening of the lumen consisted of collagen and concentrically arranged layers of elastic fibres. No thrombotic material could be observed.

Figure 2

Photomicrograph showing a segment of the superficial temporal artery with severely narrowed lumen (asterisk). The internal elastic lamina (a) is well preserved (elastica stain). The intima is considerably thickened (b) and shows tissue proliferation. There are no signs of necrosis, inflammation, or thrombus.

The patient remained stable over a 3 year follow up period and subsequent ESR and CRP were normal. She continued having diffuse body pain.

Comment

GCA is a neurological emergency which, when left unrecognised and untreated frequently leads to permanent blindness.1 ESR can be normal in 5–30% of patients,2,3 but this is an exceptionally rare diagnosis in middle aged patients.

In summary, our patient presented with a clinical picture suspicious of vasculitic occlusion of the superficial temporal artery, polymyalgia, and cough. We present this case as an illustration that this clinical picture can result from rapidly developing intimal fibrosis without any evidence of inflammation.

References

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