Porcu Cecilia, Merkel Nathalie, Fusi-Schimdhauser Tanja
Department of Internal Medicine, Ospedale Regionale di Lugano EOC, Lugano, Switzerland.
Department of Rehabilitation and Geriatrics, University of Geneva, Geneva, Switzerland.
Eur J Case Rep Intern Med. 2024 Dec 2;11(12):005015. doi: 10.12890/2024_005015. eCollection 2024.
This article describes a case of a 26-year-old female with a history of Evan's syndrome who presented with severe exertional dyspnoea and abdominal discomfort. The patient was diagnosed with chronic Budd-Chiari syndrome, a rare vascular disorder characterized by obstruction of the hepatic vein. We discuss the risk factors, the clinical manifestations, and diagnostic methods for Budd-Chiari syndrome, as well as the possible association with an underlying incomplete systemic lupus erythematosus. The importance of close follow-up and timely diagnosis for preventing disease progression and reducing mortality is emphasized. The article concludes by highlighting the need for further monitoring to identify any symptoms or signs suggesting a progression to complete lupus erythematosus.
Budd-Chiari syndrome can present as the initial manifestation of a broader autoimmune disorder such as incomplete systemic lupus erythematosus.Evan's syndrome, when associated with other thrombotic conditions like antiphospholipid syndrome, may indicate an underlying, evolving autoimmune process.Early recognition and management of incomplete lupus are crucial to prevent progression to a full-blown systemic lupus erythematosus and associated complications.
本文描述了一例26岁患有伊文氏综合征的女性病例，该患者出现严重劳力性呼吸困难和腹部不适。患者被诊断为慢性布加综合征，这是一种罕见的血管疾病，其特征为肝静脉阻塞。我们讨论了布加综合征的危险因素、临床表现、诊断方法，以及与潜在的不完全系统性红斑狼疮的可能关联。强调了密切随访和及时诊断对于预防疾病进展和降低死亡率的重要性。文章最后强调需要进一步监测，以识别任何提示进展为完全性红斑狼疮的症状或体征。
布加综合征可作为更广泛的自身免疫性疾病如不完全系统性红斑狼疮的初始表现。伊文氏综合征与抗磷脂综合征等其他血栓形成性疾病相关时，可能提示潜在的、正在发展的自身免疫过程。早期识别和处理不完全性狼疮对于预防进展为全面性系统性红斑狼疮及相关并发症至关重要。
