SEVERE HYPOFIBRINOGENEMIA IN A PATIENT WITH GIANT CELL ARTERITIS TREATED WITH TOCILIZUMAB: CASE-BASED REVIEW

Željka Kardum, Ana Šimac, Kristina Frketić Marović, Ana Marija Masle, Jasminka Milas Ahić
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Abstract

The Giant cell arteritis (GCA) is the most common form of systemic vasculitis in elderly patients. The treatment includes high doses of steroids and interleukin (IL)-6 inhibitor tocilizumab, especially in refractory or relapsing disease or in cases where there is an increased risk of steroid-related adverse events. This report discusses the case of a patient with giant cell arteritis who underwent treatment with tocilizumab for four years. The treatment was successful and resulted in clinical remission. However, four years after starting the therapy, the patient developed spontaneous hematomas on their extremities. After further investigation, it was discovered that the patient had developed thrombocytopenia and hypofibrinogenemia, which required substitution therapy. Malignancy and immune-mediated causes of hypofibrinogenemia and thrombocytopenia were ruled out. The patient experienced an extended period of hypofibrinogenemia that lasted for two months after the last dose of tocilizumab. During this time, the levels of CRP remained very low. This could be because the continued inhibition of IL-6 caused impaired hepatic synthesis of acute phase response proteins, which led to low fibrinogen and CRP levels in serum. The purpose of this case-based review is to emphasize the necessity of regular fibrinogen check-ups in GCA patients treated with tocilizumab.
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用托珠单抗治疗巨细胞动脉炎患者的严重低纤维蛋白原血症:病例回顾
巨细胞动脉炎(GCA)是老年患者最常见的全身性血管炎。治疗方法包括大剂量类固醇和白细胞介素(IL)-6抑制剂托西珠单抗,尤其是在疾病难治或复发或类固醇相关不良反应风险增加的情况下。本报告讨论了一名巨细胞动脉炎患者接受托西珠单抗治疗四年的病例。治疗取得了成功,临床症状得到缓解。然而,在开始治疗四年后,患者的四肢出现了自发性血肿。进一步检查后发现,患者出现了血小板减少和低纤维蛋白原血症,需要进行替代治疗。排除了导致低纤维蛋白原血症和血小板减少的恶性肿瘤和免疫介导的原因。在最后一次服用托西珠单抗后,患者经历了长达两个月的低纤维蛋白原血症。在此期间,CRP 水平仍然很低。这可能是因为 IL-6 的持续抑制导致肝脏合成急性期反应蛋白的能力受损,从而导致血清中纤维蛋白原和 CRP 水平较低。本病例回顾旨在强调接受托西珠单抗治疗的 GCA 患者定期进行纤维蛋白原检查的必要性。
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