Ra Seung Won, Lim Soyeoun, Cha Hee Jeong, Kim Sunyoung
Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Republic of Korea.
Department of Radiology, Ulsan University Hospital, University of Ulsan College of Medicine, Republic of Korea.
Clin Med Insights Case Rep. 2022 May 18;15:11795476221100598. doi: 10.1177/11795476221100598. eCollection 2022.
Systemic diseases can be found in neuromyelitis optica spectrum disorder (NMOSD) as a co-existing disease with paraneoplastic syndrome, sarcoidosis, or connective tissue disease. Cryptogenic organizing pneumonia (COP) in NMOSD with no evidence of these systemic disorders has rarely been reported.
We present a 75-year-old patient who showed multifocal longitudinally extensive transverse myelitis and bilateral lung lesions that was seropositive for aquaporin-4 (AQP4) antibody. The patient initially presented with chronic cough, myalgia, and severe bilateral truncal neuropathic pain, and initial chest computed tomography demonstrated multifocal consolidations with reversed halo sign involving both lobes. Since this patient was over 50 years of age, our differential diagnoses included lung cancer and sarcoidosis. Through extensive studies including lung biopsy, an idiopathic type of diffuse interstitial lung disease-cryptogenic organizing pneumonia (COP)-was finally diagnosed. The patient was treated with high-dose methylprednisolone and it was tapered with oral steroids; mycophenolate mofetil was later added to the regimen. After treatment, the severe neuropathic pain and multifocal lung consolidation resolved.
Herein, we presented a case of late-onset NMO with nonneoplastic, nonsarcoidosis, diffuse interstitial lung lesions, which is the finding of COP.
系统性疾病可在视神经脊髓炎谱系障碍（NMOSD）中作为副肿瘤综合征、结节病或结缔组织病的并存疾病被发现。在无这些系统性疾病证据的NMOSD中，隐源性机化性肺炎（COP）鲜有报道。
我们报告一名75岁患者，其表现为多灶性纵向广泛横贯性脊髓炎和双侧肺部病变，水通道蛋白4（AQP4）抗体血清学呈阳性。患者最初表现为慢性咳嗽、肌痛和严重的双侧躯干神经性疼痛，初始胸部计算机断层扫描显示双肺叶有多灶性实变伴反晕征。由于该患者年龄超过50岁，我们的鉴别诊断包括肺癌和结节病。通过包括肺活检在内的广泛检查，最终诊断为特发性弥漫性间质性肺疾病——隐源性机化性肺炎（COP）。患者接受了大剂量甲泼尼龙治疗，并逐渐减量为口服类固醇；随后在治疗方案中加入霉酚酸酯。治疗后，严重的神经性疼痛和多灶性肺实变得以缓解。
在此，我们报告了一例迟发性NMO合并非肿瘤性、非结节病性弥漫性间质性肺部病变，即COP的病例。
