Tongue necrosis secondary to giant cell arteritis

Abstract

Tongue necrosis is a rare clinical finding because of its rich vascularisation. Giant cell arteritis (GCA) is the most frequent cause of it, and when present, it is usually one side affected. We describe a patient with several months of constitutional syndrome; during that period, she develops headache followed by tongue necrosis, which lead to clinical suspicion of GCA, later confirmed by a temporal artery biopsy. Before the biopsy, she was treated with corticosteroids. We discuss this illness and tongue necrosis as a rare manifestation to consider.

Background

Giant cell arteritis (GCA) is a vasculitis that affects large and medium vessels (aorta and its subclavian and carotid branches, among others). It affects patients over 50 years. Up to 40%–60% of patients with GCA also have polymyalgia rheumatica (PMR) at the time of diagnosis. Tongue necrosis is a rare clinical finding because of its rich collateral circulation. Although it is an atypical complication of GCA, it is the most common cause of tongue necrosis, being equivalent to ocular involvement of GCA and often unilateral when present.

Case presentation

A woman in her 80s was referred from internal medicine consultations where she was being studied in the last year for constitutional syndrome. In her history record, she presented with artery hypertension, euthyroid multinodular goitre, spondyloarthrosis under follow-up by the pain unit, osteoporosis assessed by rheumatology 1 year earlier, benign paroxysmal positional vertigo, anxiety–depressive syndrome and frequent headache episodes that remitted with first WHO analgesic ladder.

During the follow-up, she had lost 10 kg of weight, of which 7 kg she had lost in the last 6 months, associated with generalised arthromyalgias and two to three times/day loose, non-bloody stools. Various tests were performed without getting conclusive data, including: colonoscopy with diverticula and a left colon tubular adenoma; and gastroscopy with an incompetent gastric cardia, a hyperplastic sessile polyp, metaplastic chronic atrophic gastritis without dysplasia and with Helicobacter pylori infection. Additionally, D-xylose test was performed with normal result. HLA-DQ2.2/DQ6 was detected with negative anti-transglutaminase, anti-LKM and anti-mitochondrial antibodies but with positive anti-gastric parietal cell antibodies. Her other test results were as follows: calprotectin 264 µg/g, faecal elastase 104 µg/g, vasoactive gut peptide 16 pg/mL, lactate dehydrogenase 272 U/L, serum iron 26 µg/dL, C reactive protein (CRP) 46 mg/L, erythrocyte sedimentation rate (ESR) 57 mm/hour and PCR for Tropheryma whipplei, which was negative. Clostridioides difficile and parasites in stools as well as fat staining were negative. Finally, she was admitted to the hospital when she weighed 35 kg, which was associated with significant malnutrition.

During her hospital stay, no diarrhoea was observed. After requestioning the patient, it was found that she had generalised arthromyalgia, although it was primarily on shoulders.

Seventy-two hours after admission, she presented with frontal headache, which was similar to her usual symptoms, with persistent shoulder pain. Twenty-four hours later, severe and sudden-onset tongue pain appeared. On physical examination, the right temporal cord was palpated, and the oropharynx had no alterations except for a 1 cm lingual lesion that evolved into necrosis 2 days later (figure 1) in the right lingual area. The neurological examination was completely normal.

Figure 1.

Right hemilingual haematoma.

Outcome and follow-up

Given the clinical suspicion of lingual ischaemia secondary to GCA, we decided to treat her immediately with 250 mg of methylprednisolone bolus for 3 days, instead of the 1 g/day standard regimen because of the low weight of the patient. A surgical debridement was also performed, without functional sequelae. Later on, the administration of prednisone at a dose of 1 mg/kg per day was continued. Ultrasound of the supra-aortic trunks and temporal arteries was performed, ruling out carotid stenosis and confirming the presence of halo sign in the left temporal artery. A biopsy of the right temporal artery was performed, which confirmed the diagnosis of GCA (figures 2 and 3). Finally, she had good response to corticosteroid treatment, achieving complete clinical remission and normalisation of CRP and ESR during follow-up.

Figure 2.

Temporal artery biopsy. The arrow shows a giant cell.

Figure 3.

Temporal artery biopsy. The arrow shows fragmentation of the internal elastic lamina.

Discussion

GCA is a vasculitis more common in women than in men, with a peak incidence between 70 and 80 years. The clinical manifestations are varied: constitutional syndrome, jaw claudication, headache, pain in the scalp and rapidly progressive loss of vision, among others are some typical ones. It is often possible to palpate a unilateral vascular cord, decrease pulse or increase sensitivity in the path of the temporal arteries¹ like in our case. GCA can be associated with PMR in up to 40%–60% of cases, and on the contrary, up to 15% of PMR cases are associated with GCA. PMR is characterised by weakness and pain in shoulders and hips, predominantly in the morning, along with stiffness.² In our patient, she had shoulder pain. Tongue necrosis is an uncommon manifestation of this disease and it usually has a poor prognosis.^{3 4}

The rich vascularisation of the tongue makes its evolution to necrosis a rare clinical manifestation. When it appears, GCA is the most frequent cause and usually has a unilateral presentation, as in our case. Other entities that can cause tongue necrosis are the use of vasopressors, disseminated intravascular coagulation, and radiotherapy or chemotherapy.⁵ Cases in which the development of tongue necrosis precipitated in patients with GCA with the use of ergotamine for the treatment of migraine have also been reported.⁵

A high index of suspicion is required to diagnose GCA when patients initially present with atypical symptoms. The diagnosis is based on clinical findings, laboratory tests like CRP and elevations, and imaging test such as temporal artery ultrasound, being the temporal artery biopsy the gold standard, although it cannot be ruled out when it is negative.^{5 6}

Tongue necrosis, like visual impairment, should be considered as an irreversible organ involvement, so the treatment consists of corticosteroids bolus at high doses of 0.5–1 g/day for 3 days, followed by 1 mg/kg/day for 2–4 weeks and then reducing the dose every 2 weeks. Conservative treatment is normally preferred, although in some cases and depending on the extent, it requires debridement of the necrotic area, as in our case, in order to preserve and improve the recovery of the remaining tissue.⁵ Methotrexate and tocilizumab can also be used as adjunctive therapy.^{7 8}

Learning points.

• Giant cell arteritis is the most frequent cause of tongue necrosis.

• Giant cell arteritis is more frequent in older people and should be evoked early in patients with an unexplained constitutional syndrome.

• Tongue necrosis should be considered as an irreversible organ involvement, so the treatment consists of corticosteroids bolus at high doses of 0.5–1 g/day for the first 3 days.

• Methotrexate and tocilizumab can also be used as adjunctive therapy.

Ethics statements

Patient consent for publication

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