Dear Editor:

Only ten cases of jaw claudication (JC) have been described in conjunction with Wegener’s Granulomatosis (WG).1-4 We present a case of WG with a clinical presentation similar to that of temporal (giant cell) arteritis (TA).

A male patient aged 63 years was examined for fever, pain and hardening of the right temporal artery and JC that had been developing over four months, with no other findings from the physical exam. The laboratory showed a GSV of 120mm/hour (normal < 20) with no microhaematuria or kidney failure. Thoracic radiograph was normal. Biopsy of the temporal artery was negative. Having ruled out other pathologies, the profile was interpreted as TA and 60mg methylprednisone/day was administered; the symptoms improved, and the corticosteroids were then gradually reduced. Six months later, the patient was taking 20mg methylprednisone daily and presented constitutional symptoms, as well as epistaxis, bilateral pulmonary nodules with cavitation and microhaematuria, with a nasal biopsy that showed necrotic granulomatous inflammation, which resulted in the diagnosis of WG. Treatment was begun with cyclophosphamide (150mg/day) and methylprednisone (60mg/day) and the symptoms improved.Antineutrophil cytoplasmic antibodies (ANCA) were positive with high titres (240AU, normal < 10) and the ELISA test revealed specificity for the proteinase 3 antigen. One year after the diagnosis, the patient was asymptomatic.

JC is an ischaemic symptom of fatigue or pain with mastication caused by the narrowing or obstruction of the facial branches of the external carotid (which irrigate muscles used in mastication), and which is present in 45% of patients with TA. Other, less common causes of JC are primary amyloidosis, polyarteritis nodosa (PAN), Churg-Strauss syndrome (CSS), Takayasu’s arteritis, GW, hairy cell leukaemia, McArdle’s disease, crioglobulinaemia associated with vasculitis, and carotid atherosclerosis.

Compromise of the temporal artery associated with JC has been shown with a very low frequency with PAN, CSS, Takayasu’s arteritis, crioglobulinaemia associates with vasculitis, primary amyloidosis and WG.2 To increase the complexity of the issue, TA may affect the kidneys and lungs just as WG can.2 In turn, TA can be associated with other types of vasculitis (such as CSS, PAN and WG),4 with rheumatoid arthritis, primary biliary cirrhosis and neoplasias. The association of WG with other types of vasculitis, such as CSS and AT, has also been described.

It is interesting to recall that among the causes of giant cells in a temporal artery biopsy, we find systemic lupus eritematous, isolated angeitis of the central nervous system, Takayasu’s arteritis, and TA. A compromised temporal artery without giant cells has also been described in some cases of systemic vasculitis such as hypersensitivity angeitis, crioglobulinaemia, CSS, WG and PAN.

There are ten patients described in the literature who had WG and an initial clinical profile compatible with TA.1-4 All of these patients were older than 60 and had JC with or without sudden loss of sight, severe headache with or without double vision, or polymyalgia rheumatica upon diagnosis. The GSV was high at the onset of symptoms in all patients. Biopsy of the temporal artery showed TA in two patients, arteritis without giant cells in four patients, and for the rest it was negative to normal, as with our patient. Within six months, the ten patients developed renal and/or pulmonary lesions characteristic of WG, with typical histologies in the biopsy or positive ANCA.

In summary, we can state that there are five different categories to describe a vasculitis-induced compromised temporal artery, which are: 1) temporal arteritis without giant cells due to multiple entities; 2) temporal arteritis with giant cells, whether caused by TA or not; 3) TA concurrent with WG or other forms of vasculitis; 4) WG with a clinical profile resembling temporal vasculitis but with a negative biopsy (our patient); and 5) TA with clinical characteristics of WG (very uncommon).

Documenting the different histological types of vasculitis that produce similar clinical manifestations emphasises the importance of obtaining a biopsy, whether diagnostic or prognostic, given that treatments may be very different.