Gomes Ferreira Sérgio, Fernandes Luís, Santos Sara, Ferreira Sofia, Teixeira Mónica
Internal Medicine, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, PRT.
Cureus. 2024 Jan 22;16(1):e52733. doi: 10.7759/cureus.52733. eCollection 2024 Jan.
Anti-synthetase syndrome (ASS), a rare immunomediated disease, is characterized by multiple signs and symptoms. Not all patients develop the entire clinical spectrum of the syndrome, as it often varies depending on the involved antibodies. In this case report, a 53-year-old non-smoking woman had complaints of fatigue and dyspnea on exertion for five weeks. The outpatient study revealed creatine kinase (CK) 351U/L, ANAs+, anti-SSa+, normal echocardiogram, and a chest X-ray suggesting imaging suspicion of SARS-CoV-2 pneumonia. Referred to the emergency department, she was hospitalized for bilateral interstitial pneumonia without respiratory failure. Three SARS-CoV-2 polymerase chain reaction tests were negative. She underwent a five-day course of dexamethasone 6mg due to suspected coronavirus disease 2019 (COVID-19) sequelae with favorable progress. About a month later, she experienced fatigue, exertional intolerance, morning cough, and Raynaud's phenomenon episodes. Anti-SARS-CoV-2 antibodies were negative, and a follow-up chest CT showed bilateral organizing pneumonia. Bronchofibroscopy and bronchoalveolar lavage with cytology suggestive of inflammatory appearance, predominantly CD8+ lymphocytes, were performed. Subsequently, positive results for anti-OJ antibodies were obtained. A diagnosis of ASS was established, and prednisolone was initiated at 60mg/day with a tapering regimen, resulting in clinical and radiological improvement. Additional therapy with azathioprine was proposed. This case is presented due to highly suggestive COVID-19 imaging changes, emphasizing the importance of a high suspicion of ASS, despite nearly exclusive pulmonary involvement, with only one isolated elevated CK value and no musculoskeletal complaints. It is also noteworthy for the association with anti-OJ antibodies, rarely identified, often presenting interstitial lung disease as an isolated manifestation.
抗合成酶综合征(ASS)是一种罕见的免疫介导性疾病,具有多种体征和症状。并非所有患者都会出现该综合征的全部临床症状,因为其症状通常因所涉及的抗体不同而有所差异。在本病例报告中,一名53岁的不吸烟女性诉说有疲劳和劳力性呼吸困难症状达五周之久。门诊检查显示肌酸激酶(CK)为351U/L,抗核抗体阳性(ANAs+),抗SSa抗体阳性,超声心动图正常,胸部X线检查提示影像学怀疑为新型冠状病毒肺炎(SARS-CoV-2肺炎)。转诊至急诊科后,她因双侧间质性肺炎住院,但无呼吸衰竭。三次新型冠状病毒2聚合酶链反应检测均为阴性。由于怀疑是2019冠状病毒病(COVID-19)后遗症,她接受了为期五天的地塞米松6mg治疗,病情进展良好。大约一个月后,她出现疲劳、运动不耐受、晨起咳嗽和雷诺现象发作。抗SARS-CoV-2抗体为阴性,胸部CT随访显示双侧机化性肺炎。进行了支气管纤维镜检查和支气管肺泡灌洗,细胞学检查提示有炎症表现,主要为CD8+淋巴细胞。随后,抗OJ抗体检测结果呈阳性。确诊为抗合成酶综合征,开始使用泼尼松龙,剂量为60mg/天,并采用逐渐减量方案,临床和影像学表现均有改善。建议加用硫唑嘌呤进行辅助治疗。之所以呈现该病例,是因为其影像学表现高度提示COVID-19,强调了高度怀疑抗合成酶综合征的重要性,尽管几乎仅累及肺部,仅有一项孤立的CK值升高且无肌肉骨骼方面的主诉。该病例还因其与抗OJ抗体相关而值得关注,抗OJ抗体很少被发现,常以间质性肺病作为孤立表现。