Strach Magdalena, Kuszmiersz Piotr, Chmura Łukasz, Korkosz Mariusz
Department of Rheumatology and Immunology, Faculty of Medicine Jagiellonian University Medical College Kraków Poland.
Department of Pathomorphology, Faculty of Medicine Jagiellonian University Medical College Kraków Poland.
Clin Case Rep. 2023 Oct 17;11(10):e7981. doi: 10.1002/ccr3.7981. eCollection 2023 Oct.
Idiopathic multicentric Castleman disease (iMCD) is challenging to diagnose due to clinical similarities with other conditions, such as Still's disease. Once diagnosed, iMCD may be effectively managed with the anti-interleukin-6 antibody siltuximab.
Here, we present the case of a 19-year-old Polish woman with persistent fever and enlarged lymph nodes and whose diagnosis remained inconclusive following initial clinical assessments and extensive laboratory analyses. The patient had subsequent complaints of joint pain and erythema which were suspicious of Still's disease and resolved with treatment with tocilizumab. Later, the progression of symptoms, such as lymphadenopathy, and elevated interleukin-6 levels were consistent with Castleman disease, leading to the diagnosis of idiopathic multicentric Castleman disease seven years after the patient first reported symptoms. Treatment with the anti-interleukin-6 antibody siltuximab resulted in complete symptom resolution and normalization of inflammatory parameters. No adverse events were reported due to treatment with siltuximab.
特发性多中心Castleman病（iMCD）因与其他疾病（如成人斯蒂尔病）临床症状相似，诊断具有挑战性。一旦确诊，iMCD可用抗白细胞介素-6抗体西妥昔单抗有效治疗。
在此，我们报告一例19岁波兰女性患者，该患者持续发热、淋巴结肿大，初始临床评估和广泛实验室分析后诊断仍不明确。患者随后出现关节疼痛和红斑，怀疑为成人斯蒂尔病，使用托珠单抗治疗后症状缓解。后来，淋巴结病等症状进展以及白细胞介素-6水平升高与Castleman病相符，导致患者首次出现症状7年后诊断为特发性多中心Castleman病。使用抗白细胞介素-6抗体西妥昔单抗治疗后症状完全缓解，炎症指标恢复正常。未报告因西妥昔单抗治疗导致的不良事件。
