CLINICAL CASE OF CHRONIC MULTIFOCAL NONBACTERIAL OSTEOMYELITIS IN A PATIENT WITH INITIALLY DIAGNOSED SYSTEMIC JUVENILE ARTHRITIS

S. Salugina, I. Nikishina, O. Borodacheva, A.N. Arefyeva, E. Gasymov
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Abstract

Systemic juvenile arthritis (sJA) is a special variant of chronic arthritis in children belonging to the spectrum of polygenic autoinflammatory diseases whose main features are persistent febrile fever, skin rashes, other systemic manifestations (serositis, lymphadenopathy, hepatosplenomegaly) as well as the presence of joint damage with the frequent development of destructive polyarthritis and osteonecrosis, high laboratory indicators of systemic inflammation either at the disease onset or delayed. The list of differentiated conditions includes many diseases such as infections, cancer, primary immunodeficiencies, monogenic autoinflammatory diseases etc. Authors represent a clinical case of a patient with sJA manifestations who was diagnosed with chronic multifocal nonbacterial osteomyelitis (CMNO), that demonstrates the great difficulties in diagnosis and the variability of the course of rheumatic diseases in childhood, which in its turn causes a delay in the timely prescription of effective therapy. Thus, patients with manifestations of a systemic inflammatory process, joint changes, especially those with an atypical course of the disease, pain in the bones and joints of a nocturnal nature, as well as an insufficient response to the therapy even long-term, are in need of conducting of a differential diagnostic search with the inclusion of CMNO, the ‘whole body’ mode magnetic-resonance tomography prescribed by a rheumatologist practitioner.
初步诊断为系统性幼年关节炎的患者患慢性多灶性非细菌性骨髓炎的临床病例
全身性幼年关节炎(sJA)是儿童慢性关节炎的一种特殊变型,属于多基因自身炎症性疾病谱,其主要特征是持续发热、皮疹、其他全身表现(血清炎、淋巴结肿大、肝脾肿大)以及关节损伤,常伴有破坏性多关节炎和骨坏死的发生,在发病时或延迟发病时全身炎症的实验室指标较高。分化病症包括许多疾病,如感染、癌症、原发性免疫缺陷、单基因自身炎症性疾病等。作者介绍了一例有 sJA 表现的患者被诊断为慢性多灶性非细菌性骨髓炎(CMNO)的临床病例,该病例显示了儿童风湿性疾病诊断的巨大困难和病程的多变性,这反过来又导致了有效治疗处方的延误。因此,有全身炎症过程表现、关节病变,尤其是病程不典型、夜间骨骼和关节疼痛,以及对治疗反应不充分(即使是长期治疗)的患者,需要进行鉴别诊断搜索,包括 CMNO、风湿病医生开具的 "全身 "模式磁共振断层扫描。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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