Athanassiou Panagiotis, Kostopoulos Markos, Tzanavari Aikaterini, Spyridis Anestis, Kostoglou-Athanassiou Ifigenia
Department of Rheumatology, St. Paul's Hospital, Thessaloniki, Greece.
Department of Endocrinology, Asclepeion Hospital, Athens, Greece.
Mediterr J Rheumatol. 2017 Dec 22;28(4):212-216. doi: 10.31138/mjr.28.4.212. eCollection 2017 Dec.
Anti-synthetase syndrome is an idiopathic inflammatory myopathy characterized by interstitial lung involvement, arthritis, Raynaud's phenomenon, mechanic's hands and fever. The case of anti-synthetase syndrome in a young female is reported. The patient presented with interstitial lung disease, initially subclinical muscle involvement, Raynaud's phenomenon, arthritis and mechanic's hands. Induction therapy was administrated, that consisted of intravenous methylprednisolone and cyclophosphamide, followed by azathioprine and prednisone with good response, and finally, complete remission. The presence of mechanic's hands in a patient with lung involvement should prompt for specific investigations such as anti-synthetase antibodies and muscle enzymes. Anti-synthetase syndrome should be aggressively managed by immunosuppressive therapy, as interstitial lung disease is a severe manifestation of the disease which may affect quality of life and life expectancy.
抗合成酶综合征是一种特发性炎性肌病，其特征为间质性肺受累、关节炎、雷诺现象、技工手和发热。本文报告了一名年轻女性抗合成酶综合征病例。该患者表现为间质性肺疾病、最初为亚临床肌肉受累、雷诺现象、关节炎和技工手。给予了诱导治疗，包括静脉注射甲泼尼龙和环磷酰胺，随后使用硫唑嘌呤和泼尼松，反应良好，最终完全缓解。肺部受累患者出现技工手应促使进行特定检查，如抗合成酶抗体和肌肉酶检查。抗合成酶综合征应通过免疫抑制治疗积极处理，因为间质性肺疾病是该疾病的严重表现，可能影响生活质量和预期寿命。
