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Year : 2023  |  Volume : 5  |  Issue : 1  |  Page : 14

Tongue necrosis and paracentral acute middle maculopathy as manifestations of giant cell arteritis

1 Department of Ophthalmology, Institute of Medical Assistance to the State Public Servant, São Paulo, Brazil
2 Department of Pathology Services, Institute of Medical Assistance to the State Public Servant, São Paulo, Brazil

Date of Submission06-Mar-2023
Date of Decision14-Mar-2023
Date of Acceptance14-Mar-2023
Date of Web Publication11-May-2023

Correspondence Address:
Nathalie Stéphanie Meneguette
180 Ministro Raul Fernandes St., Apto 1601, Rio de Janeiro, RJ 22260-040
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_13_23

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Giant cell arteritis (GCA) is the most common primary vasculitis in adults. In general, the diagnosis is straightforward when characteristic symptoms such as headaches, scalp tenderness, jaw claudication, vision problems, or other ischemic complications are present. Atypical presentations of GCA have become increasingly recognized but we report for the first time a case of GCA presenting as partial tongue necrosis and paracentral acute middle maculopathy. This case adds to the literature and emphasizes the importance of rapid recognition of these atypical changes and optical coherence tomography in the evaluation of GCA patients.

Keywords: Giant cell arteritis, paracentral acute middle maculopathy, tongue necrosis

How to cite this article:
Meneguette NS, De Castro WS, Botini LN, De Andrade EP. Tongue necrosis and paracentral acute middle maculopathy as manifestations of giant cell arteritis. Pan Am J Ophthalmol 2023;5:14

How to cite this URL:
Meneguette NS, De Castro WS, Botini LN, De Andrade EP. Tongue necrosis and paracentral acute middle maculopathy as manifestations of giant cell arteritis. Pan Am J Ophthalmol [serial online] 2023 [cited 2023 Jun 3];5:14. Available from: https://www.thepajo.org/text.asp?2023/5/1/14/376673

  Introduction Top

Giant cell arteritis (GCA) also known as temporal or cranial arteritis is a chronic, systemic vasculitis of large- and medium-sized vessels, usually affecting the over 50 years of age group with the highest incidence found in Scandinavian countries and people of northern European descent clinically can present fever, claudication of the jaw, and temporal headache. Almost two-thirds develop ocular symptoms and up to 30% have a permanent visual loss. Less common symptoms include dysphagia, cough, hearing loss, and tongue necrosis (TN).[1],[2],[3],[4],[5],[6],[7],[8],[9],[10]

TN is a rare occurrence given the tongue's extensive collateral blood supply from the lingual artery, tonsillar branch of the facial artery, and the ascending pharyngeal artery. Their common origin is the external carotid artery which goes on to become the superficial temporal artery, the site of temporal arteritis. While GCA is the main cause of TN, other less common etiologies should be excluded, such as carcinoma, embolism, drug use, radiation, syphilis, tuberculosis, and chemotherapy, among others.[1],[2],[3],[4],[5]

The most common mechanism of visual loss in GCA is anterior arteritic optic neuropathy due to vascular involvement of short posterior ciliary arteries, follow by central retinal artery occlusion (CRAO), and posterior ischemic optic neuropathy (PION), resulting from vascular involvement of the ophthalmic artery and its pial branches.[6],[7],[8],[9],[10]

Paracentral acute middle maculopathy (PAMM) is a relatively new optical coherence tomography (OCT) finding, defined by parafoveal white-grey lesions localized in the middle layer of the retina. Recent studies show an important association with GCA.

PAMM has been reported in the retinal artery or vein occlusions, either isolated or associated with systemic arterial hypertension, antiphospholipid syndrome, or cardiopulmonary bypass, among others.[8],[9],[10]

The diagnosis of GCA is clinicopathological, and the American College of Rheumatology has established the diagnosis criteria of at least 3 out of 5 positive findings:

  • Age older than 50 years at the onset of disease
  • New onset of localized headache
  • Abnormal temporal artery with tenderness or decreased pulse
  • Erythrocyte sedimentation rate (ESR) higher than 50 mm/h
  • Biopsy of the artery showing necrotizing arteritis with predominant mononuclear cell infiltrate or granulomatous process with multinucleated giant cells.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10]

Therefore, atypical forms of this disease are less studied and potentially dangerous. The goal of this article is to present a new case of TN followed by PAMM as the debut of GCA and shows the importance of multimodal analysis for the challenging diagnosis.

  Case Report Top

A 73-year-old woman presented with a history of vision loss in both eyes after 15 days of evolution of an unknown cause of painful partial TN [Figure 1] and [Figure 2]. She reports cardiac arrhythmia, and denying hypertension or diabetes. On initial examination, the best-correct visual acuity was 20/40 in the right eye (RE) and 20/400 in the left eye (LE), with a diffuse defect on confrontation visual fields in the LE. Intraocular pressures were 9 mmHg in RE and 11 mmHg in LE. Pupillary reflex and anterior biomicroscopy were normal in both eyes. Retinography and spectral-domain (SD)-OCT (Cirrus 5000, Zeiss, MN) of the macula showed multiple hyper-reflective band-like lesions limited to the level of the inner nuclear layer (INL), with gray lesions in the parafoveal position on near-infrared reflectance imaging [Figure 3] and [Figure 4]. Magnetic resonance images of the brain were normal. Elevation in both C-reactive protein and ESR (166 mg/dL and 87 mm/h, respectively.) was concordant with an acute inflammatory process. On further questioning, the patient reported headaches for the past weeks. She denied jaw claudication and arthralgias. The treatment was started with 1 g, intravenous, methylprednisolone daily for 5 days and discharged with 40 mg of oral prednisone daily and after was initiated methotrexate 15 mg/week. At the last follow-up, the best corrected visual acuity (BCAV) was 20/30 in the RE and 20/400 in LE and OCT presenting atrophy of INL [Figure 3] and [Figure 4].
Figure 1: (a-d) Evolution of clinical appearance of the tongue

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Figure 2: (a-f) Tongue Biopsy: (a-c). H and E, Grocott and Ziehl stains, respectively, at ×10 magnification. Ulceration associated with acute mucosal infiltration. Negative search for microorganisms, (d-f) H and E, and Grocott and Ziehl stains, respectively. Area of mucosal ulceration associated with acute chronic inflammatory infiltrate (at ×100 magnification). Negative microorganism scan (×10 magnification)

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Figure 3: (a) Color fundus photograph after referral to our institution. The right eye has superior branch occlusion and an acute cotton-wool stain along the inferior vascular arcade (blue arrows), (b) 5 months later showing resolution of lesions

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Figure 4: (a) Optical coherence tomography showing hyperreflectivity of the inner nuclear layer (green arrows) consistent with acute PAMM, (b and c) INL atrophy at the 2- and 5-month follow-up (yellow arrows). PAMM: Paracentral acute middle maculopathy, INL: Inner nuclear layer

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  Discussion Top

Reviewing the literature, our patient presented the same clinical findings described in later studies, and most patients with GCA-associated TN were older females.[1],[2],[3],[4],[5],[6] The incidence is 12 women to one man, with a mean age of 77 years and a mean ESR of 79 mm/h. Analyzing the pattern of necrosis, 80% of patients presented with local pain, 50% with tongue edema, and 28% progressed to ulceration. In relation to associated symptoms, only seven patients (29%) did not have a headache as an initial manifestation.[3] Ocular symptoms, such as blurred vision and sudden visual loss, occurred in 38%. All patients were treated with high doses of corticosteroids and 28% received pulse therapy with methylprednisolone. The response to corticosteroids was, in the majority (76%), satisfactory, with good healing and disease control.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10]

For adjunctive therapy, methotrexate, azathioprine, cyclophosphamide, and anti-tumor necrosis factor-alpha agents may be used. Methotrexate is the first choice as steroid-sparing drug because it has proven effective in reducing the cumulative dose of and preventing recurrences.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10]

Due to its antithrombotic and anti-inflammatory properties and favorable side effect profile, low-dose aspirin has been commonly used as an adjunctive treatment in GCA. A recent landmark study demonstrated the benefit of tocilizumab administered weekly or every other week in the treatment of GCA. This study provided convincing evidence of a steroid-sparing benefit of tocilizumab, which should improve the long-term risk-benefit profile for treating GCA.[1],[2],[3],[4],[5],[6]

Mairot et al.,[8] show that PAMM lesions are a common find in patients with GCA, with an incidence of 16.7%, when all patients with GCA are considered and 30.8% when only patients with ophthalmic involvement are considered. PAMM affected 26.1% of eyes with retinal or optic nerve involvement. As expected in GCA, the most frequent ophthalmic finding was anterior ischemic optic neuropathy affecting 72.4% of 69 affected eyes. PAMM was the second most frequent finding (26.1%). Finally, CRAO (13%) and PION (1.5%) were the least frequent.

Our patient had on SD-OCT, hyperreflective lesions limited to the level of the INL. The etiology of PAMM seems to be directly related to deep capillary plexus ischemia from GCA. Our case shows that patients with GCA may present with mild-to-severe visual loss from PAMM with ischemic injury limited to the INL.[8],[9],[10]

Although TN and PAMM are uncommon initial manifestations, they are “red flags” for early diagnosis and treatment of GCA. Our study shows as recent studies that PAMM findings on OCT can be useful for diagnosing atypical GCA.

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.

  References Top

Zaragoza JR, Vernon N, Ghaffari G. Tongue necrosis as an initial manifestation of giant cell arteritis: Case report and review of the literature. Case Rep Rheumatol 2015;2015:901795.  Back to cited text no. 1
Raveendran R, Banks S, Beck M, Albano-Aluquin S, Sabat S, Ostrov B. On the tip of the tongue: Tongue necrosis as the initial presentation of systemic vasculitis in adult and pediatric patients – Case reports and a literature review. J Fam Med Dis Prev 2016;2:047.  Back to cited text no. 2
Sobrinho RABS, de Lima KCA, Moura HC, Araújo MM, de Assis CMRB, Gouveia PADC. Tongue necrosis secondary to giant cell arteritis: A case report and literature review. Case Rep Med 2017;2017:6327437.  Back to cited text no. 3
Ramos ZA, Urquiza FI, Merino DF, Zubillaga RI, Sánchez AO. Lingual necrosis as a debut of giant cells arteritis: case report. Bibliographic review. Rev Esp Cirug Oral Maxilofac 2020;42:32-5.  Back to cited text no. 4
Dejaco C, Duftner C, Buttgereit F, Matteson EL, Dasgupta B. The spectrum of giant cell arteritis and polymyalgia rheumatica: Revisiting the concept of the disease. Rheumatology (Oxford) 2017;56:506-15.  Back to cited text no. 5
Donaldson L, Margolin E. Vision loss in giant cell arteritis. Pract Neurol 2022;22:138-40.  Back to cited text no. 6
Vodopivec I, Rizzo JF 3rd. Ophthalmic manifestations of giant cell arteritis. Rheumatology (Oxford) 2018;57:i63-72.  Back to cited text no. 7
Mairot K, Sené T, Lecler A, Philibert M, Clavel G, Hemmendinger A, et al. Paracentral acute middle maculopathy in giant cell arteritis. Retina 2022;42:476-84.  Back to cited text no. 8
Ahuja AS, El-Dairi MA, Hadziahmetovic M, Gospe SM 3rd. Paracentral acute middle maculopathy as a manifestation of giant cell arteritis. J Neuroophthalmol 2021;41:e153-6.  Back to cited text no. 9
Broyles H, Chacko J, Chancellor J, LoRusso F, Phillips PH, Mashayekhi A, et al. Paracentral acute middle maculopathy as the initial presentation of giant cell arteritis. J Neuroophthalmol 2021;41:e157-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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