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难治性结节病。

Refractory Sarcoidosis.

机构信息

Department of Medicine & Allied, Azra Naheed Medical College, Superior University, Lahore, Pakistan.

Department of Medicine, Jinnah Hospital, Allama Iqbal Medical College, Lahore, Pakistan.

出版信息

J Ayub Med Coll Abbottabad. 2023 Jul-Sep;35(3):479-481.

PMID:38404097
Abstract

A multi-organ granulomatous disease with characteristic lung manifestations, sarcoidosis generally responds well to glucocorticoid therapy but 10% of cases are refractory necessitating immunosuppressive therapy. A 58-year-old lady presented with a dry cough and progressively worsening shortness of breath for the last 12 months. On investigation, her ESR was raised but cultures, malignancy screen and TB quantiferon were negative. HRCT chest demonstrated multiple pulmonary nodules with hilar lymphadenopathy and CT guided biopsy revealed non-caseating granuloma. She was diagnosed with Pulmonary Sarcoidosis and started on oral steroids with minimal improvement. Azathioprine was added but due to gastric intolerance switched to methotrexate. Her disease however continued to worsen and infliximab was started but she developed a severe allergic reaction. She was then started on mycophenolate mofetil but her chest imaging continued to worsen. After failing prednisone, azathioprine, methotrexate, infliximab and mycophenolate mofetil, the patient was started on rituximab.

摘要

一种多器官肉芽肿性疾病,具有特征性的肺部表现,结节病通常对糖皮质激素治疗反应良好,但 10%的病例为难治性,需要免疫抑制治疗。一位 58 岁女性因干咳和进行性加重的呼吸困难 12 个月就诊。检查发现,她的 ESR 升高,但培养物、恶性肿瘤筛查和 TB quantiferon 均为阴性。胸部高分辨率 CT 显示多个肺结节伴肺门淋巴结肿大,CT 引导下活检显示非干酪样肉芽肿。她被诊断为肺结节病,开始口服类固醇治疗,但仅略有改善。加用硫唑嘌呤,但由于不耐受胃肠道反应,改为甲氨蝶呤。然而,她的疾病继续恶化,开始使用英夫利昔单抗,但她发生了严重的过敏反应。随后开始使用霉酚酸酯,但胸部影像学持续恶化。在泼尼松、硫唑嘌呤、甲氨蝶呤、英夫利昔单抗和霉酚酸酯治疗失败后,开始使用利妥昔单抗。

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