Długosz Gabriela, Masajtis-Zagajewska Anna, Nowicki Michał
Medical University of Lodz, Poland: Student Scientific Society affiliated with the Department of Nephrology, Hypertension and Kidney Transplantation.
Medical University of Lodz, Poland: 2Department of Nephrology, Hypertension and Kidney Transplantation.
Pol Merkur Lekarski. 2022 Oct 21;50(299):306-308.
Antiphospholipid syndrome (APS) is defined as presence of antiphospholipid antibodies along with hypercoagulable events. Renal involvement in APS usually manifests as thromboembolic complications observed in large blood vessels or small intrarenal vessels (APS nephropathy). We report a rare case of glomerulitis associated with APS and being characterised by features different from typical APS nephropathy.
A 23-years-old patient was admitted to the nephrology department with steroid-sensitive, steroid-dependent nephrotic syndrome secondary to minimal change disease, first diagnosed at the age of 18 months. Subsequent thromboembolic events, such as deep vein thrombosis, unilateral thrombosis of the popliteal artery, and pulmonary embolism, led to the diagnosis of antiphospholipid syndrome. The patient also had a history of acute kidney injury. On the day of admission, the patient had normal renal function and was taking an increased dose of prednisone owing to nephrotic syndrome relapse. In the past, attempts to reduce the dosage of glucocorticoids were made using cyclophosphamide, cyclosporin A, mofetil mycophenolate, and their combinations. It rendered ineffective as nephrotic syndrome relapses occurred. The patient's eligibility for the rituximab treatment was established in a series of diagnostic tests which excluded any contraindications. No adverse effects were observed during or after intravenous infusion of the drug.
The case emphasizes a rare renal clinical manifestation of APS in nephrologist's practice. The combination of anti-CD20 monoclonal antibody and anticoagulants is highly likely to be the optimal solution for this problem.
抗磷脂综合征（APS）定义为抗磷脂抗体的存在以及高凝事件。APS的肾脏受累通常表现为在大血管或肾内小血管中观察到的血栓栓塞并发症（APS肾病）。我们报告一例罕见的与APS相关的肾小球炎病例，其特征与典型的APS肾病不同。
一名23岁患者因微小病变病继发的激素敏感型、激素依赖型肾病综合征入住肾病科，该患者18个月大时首次确诊。随后发生的血栓栓塞事件，如深静脉血栓形成、腘动脉单侧血栓形成和肺栓塞，导致抗磷脂综合征的诊断。该患者还有急性肾损伤病史。入院当天，患者肾功能正常，因肾病综合征复发正在增加泼尼松剂量。过去，曾尝试使用环磷酰胺、环孢素A、霉酚酸酯及其联合用药来减少糖皮质激素剂量，但由于肾病综合征复发而无效。通过一系列排除任何禁忌证的诊断测试确定了该患者使用利妥昔单抗治疗的资格。静脉输注该药物期间及之后未观察到不良反应。
该病例强调了APS在肾病科临床实践中的一种罕见肾脏表现。抗CD20单克隆抗体与抗凝剂联合使用很可能是解决该问题的最佳方案。
