Bellou Anna Maria, Bös Dominik, Kukuk Guido, Gembruch Ulrich, Merz Waltraut Maria
Department of Obstetrics and Prenatal Medicine.
Department of Internal Medicine.
Medicine (Baltimore). 2018 Apr;97(17):e0401. doi: 10.1097/MD.0000000000010401.
Lupus enteritis is a rare, severe complication of systemic lupus erythematosus (SLE). We report of a patient who presented with enteritis as manifestation of new-onset SLE during the first trimester of pregnancy.
The 23-year nulliparous patient was admitted to a district hospital with abdominal pain, nausea, vomiting and bloody diarrhea at a gestational age (GA) of 10 weeks. Her symptoms improved with symptomatic treatment and she was discharged a few days later. At 15 weeks' of gestation she was readmitted. Her lab results revealed mild anemia and thrombocytopenia. Ascites, renal failure and proteinuria developed. An infectious cause was suspected, but stool samples and urine cultures were negative. Diagnostic work-up included abdominal ultrasound, gastro- and sigmoidoscopy, magnetic resonance imaging (MRI), and diagnostic laparoscopy. Ultrasound and MRI revealed dilated, fluid-filled small bowel loops, and increased colonic wall diameters. Mucosal edema and petechiae were detected by sigmoidoscopy, and histopathologic examination of the biopsies revealed erosive inflammation. Due to progressive deterioration she was transferred to our center. In addition to ascites, pleural and pericardial effusions had developed.
Diagnosis of SLE was finally established at GA 16 after an autoimmune workup revealed positive antinuclear, anti- Sm, anti-dsDNA and anti-U1RNP antibodies. An interdisciplinary team was set up for her management. She was commenced on corticosteroids; response was only partial and necessitated addition of cyclosporine. The further clinical course was complicated by anemia, chest wall shingles, hypertension, and progressive cervical shortening. Serial ultrasound and Doppler examinations revealed notching of the uterine arteries with raised pulsatility indices and fetal growth restriction.
At GA 35 abdominal pain reoccurred; a decision for delivery was taken. An apparently healthy fetus was delivered by cesarian section with good Apgar scores and pH (2100g, 9. percentile). The postoperative / postnatal course was unremarkable.
New-onset SLE during pregnancy is rare, as is lupus enteritis. To our knowledge, our case is the first report of a combination of both.
Diagnostic delay occurred a result of symptom overlap and limitations in diagnostic imaging. Interdisciplinary teamwork resulted in successful outcome for both, mother and fetus.
狼疮性肠炎是系统性红斑狼疮(SLE)一种罕见的严重并发症。我们报告了一名患者,其在妊娠早期以肠炎为新发SLE的表现。
这位23岁未生育的患者因在孕10周时出现腹痛、恶心、呕吐和血性腹泻入住一家区级医院。经对症治疗后症状改善,几天后出院。妊娠15周时她再次入院。实验室检查结果显示轻度贫血和血小板减少。出现了腹水、肾衰竭和蛋白尿。怀疑有感染原因,但粪便样本和尿培养均为阴性。诊断性检查包括腹部超声、胃肠镜和乙状结肠镜检查、磁共振成像(MRI)以及诊断性腹腔镜检查。超声和MRI显示小肠肠袢扩张、充满液体,结肠壁直径增加。乙状结肠镜检查发现黏膜水肿和瘀点,活检组织病理检查显示糜烂性炎症。由于病情逐渐恶化,她被转至我们中心。除腹水外,还出现了胸腔和心包积液。
在妊娠16周时,经过自身免疫检查发现抗核抗体、抗Sm抗体、抗双链DNA抗体和抗U1RNP抗体呈阳性,最终确诊为SLE。为其治疗组建了一个跨学科团队。开始使用皮质类固醇治疗;反应仅部分有效,因此需要加用环孢素。进一步的临床过程因贫血、胸壁带状疱疹、高血压和宫颈逐渐缩短而复杂化。系列超声和多普勒检查显示子宫动脉切迹,搏动指数升高以及胎儿生长受限。
妊娠35周时再次出现腹痛;决定进行分娩。通过剖宫产娩出一名外观健康的胎儿,阿氏评分和pH值良好(2100g,第9百分位数)。术后/产后过程顺利。
妊娠期新发SLE罕见,狼疮性肠炎同样罕见。据我们所知,我们的病例是两者合并的首例报告。
由于症状重叠和诊断成像的局限性导致诊断延迟。跨学科团队合作使母亲和胎儿均获得成功结局。