以急性肾功能衰竭为首发表现的系统性红斑狼疮八例临床分析

目的 了解以急性肾功能衰竭（ARF）为首发表现的系统性红斑狼疮（SLE）的临床特征，提高对该病的诊治水平。方法 收集上海长征医院1995年1月至2006年4月收治随访的以ARF为首发表现的SLE 8例，结合文献复习，对其临床表现、治疗方法、预后进行分析。结果 ①SLE急性肾功能减退的症状以少尿型为主，伴随症状及并发症重且多。②在实验室检查中自细胞及血红蛋白较低，补体下降明显，肾脏病理以狼疮肾炎（LN）Ⅳ型、Ⅳ＋Ⅴ型为主。③治疗上以大剂量激素加环磷酰胺（CTX）冲击治疗为主，病情危重时可予以缓慢连续超滤（SCUF）、连续静脉静脉血液透析滤过（CVVHDF）、维持性血液透析等治疗。结论 急性肾功能衰竭可以出现在SLE病程中，但也可以是SLE的首发症状。大剂量激素和CTX冲击治疗有明显疗效，对反应不佳的可行SCUF、CAVHDF和血液透析治疗，能明显改善患者的预后。     

Pre-eclampsia with acute heart failure postpartum as primary manifestation of systemic lupus erythematosus

Pre-eclampsia occurs in 2-5% of pregnancies of healthy women. Here, we present a rare case of pre-eclampsia with overt acute heart failure, which was the primary manifestation of systemic lupus erythematosus with cardiac and renal involvement.     

Cardiac tamponade in the early postpartum period as the presenting and predominant manifestation of systemic lupus erythematosus

Pericarditis is a common manifestation of systemic lupus erythematosus (SLE), however, tamponade seems to be extremely rare. A review of the English literature revealed 12 such cases and in 5 the tamponade was the presenting symptom of the disease. We describe an additional patient. She developed pericardial tamponade one week after delivery of a healthy child, and subsequently was diagnosed to have SLE. The effect of pregnancy and the postpartum period on SLE is discussed.     

Irreversible acute renal failure in idiopathic nephrotic syndrome

In five adult patients (aged 44 to 74 years) with idiopathic nephrotic syndrome, irreversible acute renal failure developed. Prior renal disease, associated systemic illness or occlusion of major renal vasculature was not present. All patients continued to excrete large amounts of proteins (8.6 to 15 g/24 hours) despite a minimal glomerular filtration rate and severe oliguria. One patient died after five months without recovering renal function. Four patients have required hemodialysis for a period of 12 to 58 months. The failure to recover renal function could not be explained by the light microscopic findings. It is suggested that the irreversibility of the renal failure may be related to either permanent alterations in renal blood flow or ultrastructural changes, or to both. Clinically, adult patients In whom acute renal failure develops during the course of idiopathic nephrotic syndrome seem to have a grave prognosis. Protracted oliguria or irreversible renal failure can be expected to occur.     

Glomerular thrombosis: an unusual cause of renal failure in systemic lupus erythematosus

The authors report an unusual case of acute renal failure occurring in a patient with systemic lupus erythematosus and antiphospholipid antibodies. Kidney biopsy revealed glomerular thrombosis, in the absence of glomerulonephritis. The authors stress the clinical and biological signs that suggest the thrombotic nature of kidney failure in lupus patients.     

Preclinical and early systemic lupus erythematosus

The challenge of early diagnosis and treatment is a timely issue in the management of systemic lupus erythematosus (SLE), as autoimmunity starts earlier than its clinical manifestations. Hence, growing efforts for stratification of patients according to the individual risk of developing specific clinical manifestations and/or predicting a better response to a given treatment have led to the proposal of several biomarkers, which require validation for use in clinical practice. In this viewpoint, we aim at distinguishing and discussing the features and the approach to asymptomatic immunological abnormalities potentially heralding the development of SLE, defined as preclinical lupus, and clinical manifestations consistent with SLE not yet fulfilling classification criteria, defined as early lupus. In case of preclinical SLE, careful surveillance using available screening tools is paramount, while patients with early lupus deserve an appropriate and timely diagnosis and, consequently, a proper treatment including hydroxychloroquine as the anchor drug.     

Malignant lymphoma with acute renal failure and nephrotic syndrome

A case of non-Hodgkin's lymphoma with nephrotic syndrome and acute renal failure is described. A 60-year-old Japanese male was admitted to our hospital in November, 1988, because of lymphadenopathy, fever, night sweat, weight loss. On physical examination, lymphadenopathy was present in both cervical, submandibular, supraclavicular, axillary and inguinal regions. The leukocyte count was 9,700/microliters with 85% neutrophils and 2% atypical lymphocytes. Renal function was normal. Lymph node biopsy showed non-Hodgkin's lymphoma of diffuse, large cell type. Immunohistologic examination showed T cell type. A few days later, he fell into acute renal failure. After systemic chemotherapy, he showed prompt improvement in renal function. Echo and computerized tomography (CT) of abdomen revealed no compression of the ureters and bladder. Renal biopsy findings suggested mesangium proliferative glomerulonephritis without invasion of tumor cells. Our case seemed to be rare and was compared with previous reports.     

肾病综合征合并急性肾衰诱因分析及治疗体会

报告１２例原发性肾病综合征合并急性肾功能衰竭的病例，经大剂量激素冲击疗法，环磷酰胺及血透、血滤等治疗后，９例肾功能恢复，尿蛋白消失或减少。文章分析了ＮＳ引起ＡＲＦ的肾外及肾内病因，指出要高度警惕以ＡＲＦ为首发症状的ＮＳ患者，对治疗及相关因素进行了讨论。