Umer Anum, Bhatti Shoaib, Jawed Shafaq
Internal Medicine, The Indus Hospital, Karachi, PAK.
Pediatrics, National Institute of Child Health, Karachi, PAK.
Cureus. 2018 Oct 22;10(10):e3478. doi: 10.7759/cureus.3478.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting multiple systems by the process of inflammation and formation of auto-antibodies. When it presents in childhood, it is referred to as childhood systemic lupus erythematosus (cSLE). Cardiac tamponade is a rare but potentially lethal complication of cSLE, even rarer as an initial presentation. Sub-acute cardiac tamponade (medical tamponade) is a non-emergent type of cardiac tamponade which develops slowly over time and does not necessarily present with acute distress. We present the case of an 11-year-old girl who presented to the emergency department with complaints of intermittent fever, periorbital puffiness, abdominal distension, and swelling on the hands and feet. She was not in any acute distress but was vitally unstable. Cardiovascular examination revealed muffled heart sounds. Chest examination further revealed decreased breathing sounds on the left side with dull notes on percussion. Abdominal examination revealed positive shifting dullness with a distended abdomen. Blood investigations were ordered which revealed anemia and thrombocytopenia. Chest X-ray showed an enlarged cardiac silhouette. Urine detailed report showed proteinuria and hematuria. Further investigations revealed the autoimmune root of the disease. Echocardiography was ordered which showed a large collection of fluid around the posterior aspect of heart with the concomitant collapse of atrial chambers suggestive of cardiac tamponade. A diagnosis of sub-acute cardiac tamponade secondary to childhood SLE was made. The patient was started on pulse therapy of methylprednisolone followed by a low-dose regime of mycophenolate mofetil. The patient was also provided with positive pressure ventilation, hemodialysis, and invasive cardiovascular monitoring along with the instillation of intravenous fluid supplements. To our knowledge, cases of sub-acute cardiac tamponade as the only and early clinical manifestation in childhood SLE are very rare.
系统性红斑狼疮(SLE)是一种慢性自身免疫性疾病,通过炎症过程和自身抗体形成影响多个系统。当它在儿童期出现时,被称为儿童系统性红斑狼疮(cSLE)。心脏压塞是cSLE罕见但可能致命的并发症,作为初始表现则更为罕见。亚急性心脏压塞(医源性压塞)是一种非紧急类型的心脏压塞,随着时间的推移缓慢发展,不一定表现为急性窘迫。我们报告一例11岁女孩,她因间歇性发热、眶周肿胀、腹胀以及手脚肿胀到急诊科就诊。她没有任何急性窘迫,但生命体征不稳定。心血管检查发现心音减弱。胸部检查进一步发现左侧呼吸音减弱,叩诊呈浊音。腹部检查发现移动性浊音阳性且腹部膨隆。进行了血液检查,结果显示贫血和血小板减少。胸部X线显示心脏轮廓增大。尿液详细报告显示蛋白尿和血尿。进一步检查揭示了该疾病的自身免疫根源。进行了超声心动图检查,结果显示心脏后方有大量积液,同时心房腔塌陷,提示心脏压塞。诊断为儿童SLE继发的亚急性心脏压塞。患者开始接受甲泼尼龙脉冲治疗,随后采用低剂量霉酚酸酯治疗方案。患者还接受了正压通气、血液透析和有创心血管监测,并静脉输注液体补充剂。据我们所知,亚急性心脏压塞作为儿童SLE唯一的早期临床表现的病例非常罕见。