Cabaleiro-Raña Noelia, Álvarez-Reguera Carmen, Cervantes Pérez Evelin Cecilia, Romar de Las Heras Lucía, Santos-Álvarez Diego, Álvarez Álvarez Carlos, Romero-Yuste Susana
Rheumatology Department, University Hospital Complex of Pontevedra, Pontevedra, Spain.
Patology Department, University Hospital Complex of Pontevedra, Pontevedra, Spain.
Rheumatol Int. 2025 Sep 12;45(10):226. doi: 10.1007/s00296-025-05969-2.
Lingual necrosis is a rare but serious complication of giant cell arteritis (GCA). Diagnosis can be difficult due to its atypical presentation, particularly when it occurs without the usual GCA symptoms. We present the case of a 59-year-old female with a history of migraines and smoking, who developed severe tongue pain and neck discomfort. Glucocorticoid therapy was promptly initiated due to clinical suspicion of GCA. However, when the steroid dosage was reduced, the patient's symptoms worsened despite initial improvement. Tocilizumab was subsequently introduced, and by discharge, there was a marked reduction in tongue swelling and evidence of progressive healing. A review of 55 reported cases of lingual necrosis, including ours, revealed an average age of 77.8 years and a female predominance. Hypertension was the most common cardiovascular risk factor, and 32.7% of cases presented with tongue necrosis as the initial manifestation. Most patients had their diagnosis confirmed by temporal artery biopsy, however in other cases, imaging verified the diagnosis. Even though glucocorticoids were still the primary treatment, 13 patients needed other immunosuppressive medications. Tocilizumab has demonstrated promising results in reducing glucocorticoid exposure and improving remission rates. This case highlights the importance of considering GCA in the differential diagnosis of lingual necrosis, even in younger patients or those with atypical presentations. To avoid irreparable consequences, early detection and timely treatment beginning are essential. Tocilizumab may be used as an effective therapeutic option for cases that don't respond to glucocorticoids.
舌坏死是巨细胞动脉炎(GCA)一种罕见但严重的并发症。由于其非典型表现,诊断可能困难,尤其是在无常见GCA症状的情况下发生时。我们报告一例59岁有偏头痛和吸烟史的女性,她出现了严重的舌痛和颈部不适。因临床怀疑GCA迅速开始糖皮质激素治疗。然而,当激素剂量减少时,尽管最初有所改善,但患者症状恶化。随后使用了托珠单抗,出院时舌肿胀明显减轻且有逐渐愈合迹象。对包括我们病例在内的55例报告的舌坏死病例回顾显示,平均年龄为77.8岁,女性居多。高血压是最常见的心血管危险因素,32.7%的病例以舌坏死为首发表现。大多数患者通过颞动脉活检确诊,然而在其他病例中,影像学检查证实了诊断。尽管糖皮质激素仍是主要治疗方法,但13例患者需要其他免疫抑制药物。托珠单抗在减少糖皮质激素暴露和提高缓解率方面已显示出有前景的结果。该病例强调了在舌坏死鉴别诊断中考虑GCA的重要性,即使在年轻患者或有非典型表现的患者中。为避免不可挽回的后果,早期检测和及时开始治疗至关重要。托珠单抗可作为对糖皮质激素无反应病例的有效治疗选择。