肠系膜脂膜炎(MP),也被称为硬化性肠系膜炎,是一种罕见的特发性疾病,其特征是肠系膜的慢性炎症和纤维化。虽然在MP患者中,由于淋巴引流阻塞引起的少量乳糜性腹水并不罕见,大量腹水是一种罕见的并发症。此外,蛋白质丢失性肠病(PLE),一种罕见的肠道疾病,即未补偿的血浆蛋白损失,可发生在MP患者中。据我们所知,本研究是第一个在大阪医学和药科大学医院(大阪,日本)2023年3月。大约5年前,病人注意到全身水肿,短暂性腹痛、发烧和体重减轻,经腹腔穿刺和小肠内镜检查诊断为乳糜性腹水和PLE。尽管最初的泼尼松龙(20mg/天)给药改善了水肿的逐渐和不受控制的液体积聚。计算机断层扫描显示气胸,双侧大量胸腔积液,和肺炎。尽管进行了广泛的抗生素治疗[伏立康唑(300毫克,两次/天),氨苄西林/舒巴坦(3gx4/天),和万古霉素(1,000mgx2/天)],患者在1个月后死于呼吸衰竭.尸检显示大量乳糜腹水,胸腔积液和肠系膜中增厚和钙化结节的存在。组织病理学检查显示弥漫性脂肪坏死伴纤维硬化,肠系膜内钙化和淋巴细胞浸润。因此,对MP进行了明确诊断.本病例强调了将MP作为并发乳糜性腹水病例的鉴别诊断的重要性。腹痛患者的胸腔积液和PLE,发烧和减肥。
Mesenteric panniculitis (MP), also known as sclerosing mesenteritis, is a rare idiopathic condition characterised by chronic inflammation and fibrosis in the mesentery. While small amounts of chylous
ascites due to lymph drainage obstruction are not rare in patients with MP, massive
ascites is a rare complication. Moreover, protein-losing enteropathy (PLE), a rare intestinal condition of uncompensated plasma protein loss, can occur in patients with MP. To the best of our knowledge, the present study is the first to report MP with massive chylous pleural effusion and PLE in a 56-year-old male presenting with dyspnoea at Osaka Medical and Pharmaceutical University Hospital (Osaka, Japan) in March 2023. Approximately 5 years prior, the patient noticed systemic oedema, transient abdominal pain and fever and weight loss, and was diagnosed with chylous
ascites and PLE by abdominal paracentesis and endoscopic examination of the small intestine. Although initial prednisolone (20 mg/day) administration improved the oedema gradual and uncontrolled fluid buildup was observed. Computed tomography revealed pneumothorax, bilateral massive pleural effusion, and pneumonia. Despite extensive antibiotic therapy [voriconazole (300 mg, twice/day), Ampicillin/Sulbactam (3 g x 4/day), and Vancomycin (1,000 mg x 2/day)], the patient succumbed to respiratory failure 1 month later. Autopsy revealed massive chylous
ascites, pleural effusion and the presence of thickened and calcified nodules in the mesentery. Histopathological examination showed diffuse fat necrosis with fibrosclerosis, calcification and lymphocytic infiltration within the mesentery. Therefore, a definitive diagnosis of MP was made. The present case highlighted the importance of considering MP as a differential diagnosis in cases of concurrent chylous
ascites, pleural effusion and PLE in patients with abdominal pain, fever and weight loss.