神经性类风湿病诱发的肩胛骨摇摆:英夫利西单抗治疗在应对多方面挑战中的疗效。

IF 0.9 Q4 RHEUMATOLOGY Modern rheumatology case reports Pub Date : 2024-05-23 DOI:10.1093/mrcr/rxae030
Richa Purohit, Ravi Shahu Khal, Kathleen McCabe, Neha Bhanusali, Maria Farooq, Shazia Beg
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摘要

肉样瘤病是一种主要影响呼吸系统和淋巴系统的全身性肉芽肿性疾病,很少会单独或与其他全身症状同时表现为神经肉样瘤病。在此,我们描述了一例 45 岁男性患者的病例,该患者有反复发作的鼻窦炎病史,抗生素治疗无效,因鼻窦充血和吞咽困难到急诊科就诊。临床检查发现他患有左下运动神经元面瘫和颌下腺唾液腺肿大。尽管各种抗体检测结果均为阴性,但患者的血管紧张素转换酶水平却升高至 83 nmol/kg/min。此外,胸部计算机断层扫描显示双侧肺门和纵隔淋巴结肿大,结果与肉样瘤病一致。耳鼻喉科对吞咽困难进行了评估,确诊为左侧声带麻痹。传染病检查阴性后,颌下腺唾液腺活检证实了肉样瘤病。使用霉酚酸酯和口服类固醇治疗后,涎腺肿胀、吞咽困难和面瘫症状逐渐好转。然而,左肩疼痛的恶化促使他进行了进一步检查,复查时发现左肩胛骨呈翼状。颈椎磁共振成像(MRI)显示,左侧脊髓背侧C5水平有6毫米的高密度,提示可能是神经肉芽肿病与脱髓鞘疾病。随后,医生给患者开了抗肿瘤坏死因子α抑制剂英夫利昔单抗。在开始使用英夫利昔单抗治疗六个月后,对颈椎进行了核磁共振成像检查,结果显示病变已经消退。患者表示症状有所改善,尤其是肩部疼痛减轻,左肩胛骨翼状突起有所改善,这也为治疗结果提供了佐证。该病例强调了贝尔氏麻痹和声带麻痹在同一患者身上的不寻常并发症,以及神经肉芽肿病对肩胛骨折翼的潜在影响。此外,该病例还揭示了神经肉芽肿病对英夫利西单抗(Infliximab)治疗的积极反应。
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Neurosarcoidosis-Induced Winging Scapula: Efficacy of Infliximab Treatment in Addressing Multifaceted Challenges.

Sarcoidosis, a systemic granulomatous disease primarily affecting the respiratory and lymphatic systems, can rarely manifest as neurosarcoidosis either in isolation or alongside other systemic symptoms. Here, we describe the case of a 45-year-old male with a history of recurrent sinusitis refractory to antibiotics, who presented to the emergency department with sinus congestion and dysphagia. Clinical examination revealed left lower motor neuron facial palsy and enlarged submandibular salivary glands. Despite obtaining negative results from various antibody panels, the patient exhibited elevated Angiotensin Converting Enzyme levels of 83 nmol/kg/min. Additionally, computed tomography chest scans revealed bilateral hilar and mediastinal lymph node enlargement, findings consistent with sarcoidosis. Otorhinolaryngology evaluation for dysphagia confirmed left vocal cord palsy. Following a negative infectious disease workup, submandibular salivary gland biopsy confirmed sarcoidosis. Treatment with mycophenolate mofetil and oral steroids led to gradual improvement in salivary gland swelling, dysphagia, and facial palsy. However, worsening left shoulder pain prompted further investigation, revealing winging of the left scapula on repeat examination. Magnetic resonance imaging (MRI) of the cervical spine revealed a six mm hyperintensity in the left dorsal cord at the C5 level, suggesting possible neurosarcoidosis vs. demyelinating disease. Subsequently, the patient was prescribed anti-tumor necrosis factor alpha inhibitor infliximab. Subsequent MRI of the cervical spine, conducted six months after initiating Infliximab therapy, indicated resolution of the lesions. This positive outcome was supported by the patient's report of symptom improvement, notably reduced shoulder pain and improvement in left scapular winging. This case underscores the unusual co-occurrence of Bell's palsy and vocal cord palsy in the same patient, along with the potential contribution of neurosarcoidosis to the winged scapula. Additionally, it sheds light on the positive response of neurosarcoidosis to Infliximab therapy.

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